https://proteopedia.org/api.php?action=feedcontributions&feedformat=atom&user=Alexandra+Helleux 2026-10-06T07:26:22Z User contributions MediaWiki 1.43.8 https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2526049 2016-01-29T21:49:09Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], [http://www.rcsb.org/pdb/protein/Q9H4I9 EMRE]&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystalised in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discovery of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723784/Secondary_structure/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix (in pink), beta sheets (in orange), turns (in purple) and loops (in white). When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See also ==<br /> <br /> :http://www.ncbi.nlm.nih.gov/pubmed/24336167<br /> :http://www.ncbi.nlm.nih.gov/pubmed/23101630<br /> :http://www.ncbi.nlm.nih.gov/pubmed/24503055<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2526047 2016-01-29T21:47:00Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], [http://www.rcsb.org/pdb/protein/Q9H4I9 EMRE]&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723784/Secondary_structure/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix (in pink), beta sheets (in orange), turns (in purple) and loops (in white). When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See also ==<br /> <br /> :http://www.ncbi.nlm.nih.gov/pubmed/24336167<br /> :http://www.ncbi.nlm.nih.gov/pubmed/23101630<br /> :http://www.ncbi.nlm.nih.gov/pubmed/24503055<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2526039 2016-01-29T21:28:03Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], [http://www.rcsb.org/pdb/protein/Q9H4I9 EMRE]&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723784/Secondary_structure/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix(in pink), beta sheets (in orange), turns (in purple) and loops (in white). When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See also ==<br /> <br /> :http://www.ncbi.nlm.nih.gov/pubmed/24336167<br /> :http://www.ncbi.nlm.nih.gov/pubmed/23101630<br /> :http://www.ncbi.nlm.nih.gov/pubmed/24503055<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2526037 2016-01-29T21:26:13Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], [http://www.rcsb.org/pdb/protein/Q9H4I9 EMRE]&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723784/Secondary_structure/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix(in pink), beta sheets (in orange) and loops (in white). When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See also ==<br /> <br /> :http://www.ncbi.nlm.nih.gov/pubmed/24336167<br /> :http://www.ncbi.nlm.nih.gov/pubmed/23101630<br /> :http://www.ncbi.nlm.nih.gov/pubmed/24503055<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525932 2016-01-29T18:19:21Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], [http://www.rcsb.org/pdb/protein/Q9H4I9 EMRE]&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See also ==<br /> <br /> :http://www.ncbi.nlm.nih.gov/pubmed/24336167<br /> :http://www.ncbi.nlm.nih.gov/pubmed/23101630<br /> :http://www.ncbi.nlm.nih.gov/pubmed/24503055<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525927 2016-01-29T18:00:50Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], [http://www.rcsb.org/pdb/protein/Q9H4I9 EMRE]&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525926 2016-01-29T17:59:31Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], [http://www.rcsb.org/pdb/protein/Q9H4I9 EMRE]&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525925 2016-01-29T17:57:06Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;[http://www.rcsb.org/pdb/gene/MICU2 MICU 2],[http://www.rcsb.org/pdb/gene/MCU MCU], EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525910 2016-01-29T13:45:41Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. <br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525909 2016-01-29T13:44:44Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. Calcium is involved in signalling pathways in cells and because of that it plays a lot of important roles throughout the body (muscle contraction, cell growth, cell movement...)<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525908 2016-01-29T13:40:59Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a &lt;scene name='72/723784/Homooligomer/1'&gt;homooligomer&lt;/scene&gt;.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525907 2016-01-29T13:32:57Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. It is a helix loop helix structural domain that means that there are 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1, residues concerned are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525906 2016-01-29T13:30:49Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell. This function is necessary for the activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525905 2016-01-29T13:27:37Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane and so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation and separates into multiple homooligomers. This leads to the opening of the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525904 2016-01-29T13:23:06Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains described after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525903 2016-01-29T13:20:33Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium concentration is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). The recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains describe after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux/Sandbox1138&diff=2525902 2016-01-29T13:17:17Z

<p>Alexandra Helleux: New page: ==Structure of MICU1== &lt;StructureSection load=&#039;4NSC&#039; size=&#039;340&#039; side=&#039;right&#039; caption=&#039;Caption for this structure&#039; scene=&#039;&#039;&gt; MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mi...</p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). Recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel.<br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains describe after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains :<br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions<br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do<br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=User:Alexandra_Helleux&diff=2525901 2016-01-29T13:15:30Z

<p>Alexandra Helleux: </p> <hr /> <div>*[[User:Alexandra Helleux/Sandbox1138]]<br /> * Full Real Name:Alexandra Helleux<br /> <br /> * Position:Student<br /> <br /> * Institution (NO ABBREVIATIONS):Ecole Supérieure de Biotechnologie de Strasbourg<br /> <br /> * City, State/Province, Country:Strasbourg, Alsace, France<br /> <br /> * Field of Expertise or Study: Ingeniering school, BAC+3</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525900 2016-01-29T13:02:52Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). Recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains describe after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- &lt;scene name='72/723172/C-helix_region/3'&gt;C-helix region&lt;/scene&gt; (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do <br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525899 2016-01-29T13:01:00Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> == History ==<br /> MICU1 was identified as an essential element of mitochondrial calcium uptake in 2010. It was crystallise in the presence of calcium chloride and methyl-pentanediol (MPD). Recent discover of this protein doesn’t permit to have more information about its evolutionary conservation.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> MICU 1 is a ~54kDa transmembrane protein composed by an amino-terminal mitochondrial targeting sequence, a transmembrane helix and a cytosolic C-terminus which contain different important domains describe after.<br /> <br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- C helix region (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do <br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525898 2016-01-29T12:59:29Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- C helix region (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- &lt;scene name='72/723172/Polybasic_region/1'&gt;Polybasic region&lt;/scene&gt; : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do <br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525593 2016-01-28T15:58:42Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure of MICU1==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- C helix region (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- Polybasic region : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do <br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525592 2016-01-28T15:57:52Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> == Structural highlights ==<br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- C helix region (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- Polybasic region : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do <br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525577 2016-01-28T15:38:37Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> &lt;table&gt;&lt;tr&gt;&lt;td colspan='2'&gt;<br /> &lt;/td&gt;&lt;/tr&gt;&lt;tr id='ligand'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Ligand|Ligands:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Ca 2+&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='related'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Related_structure|Related:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;MICU 2, MCU, EMRE&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='gene'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;[[Gene|Gene:]]&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;CBARA, chromosome 10 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&amp;srchmode=5&amp;id=9606 Homo sapiens])&lt;/td&gt;&lt;/tr&gt;<br /> &lt;tr id='activity'&gt;&lt;td class=&quot;sblockLbl&quot;&gt;&lt;b&gt;Activity:&lt;/b&gt;&lt;/td&gt;&lt;td class=&quot;sblockDat&quot;&gt;Regulator of Ca 2+ uptake in the mitochondria&lt;/td&gt;&lt;/tr&gt;<br /> &lt;/table&gt;<br /> <br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == Structural highlights ==<br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- C helix region (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- Polybasic region : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do <br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525560 2016-01-28T15:15:43Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == Structural highlights ==<br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- C helix region (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- Polybasic region : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;<br /> :https://www.researchgate.net/publication/260152737_Structural_and_mechanistic_insights_into_MICU1_regulation_of_mitochondrial_calcium_uptake<br /> :http://ghr.nlm.nih.gov/gene/MICU1<br /> :http://www.rcsb.org/pdb/home/home.do <br /> :http://www.uniprot.org/uniprot/Q9BPX6</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525559 2016-01-28T15:14:08Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> MICU 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> [http://www.uniprot.org/uniprot/Q9BPX6 Uniprot MICU1]<br /> <br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == Structural highlights ==<br /> In absence of Ca2+, MICU1 is an homohexamer with a &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt; composed by several alpha helix, beta sheets and loops. When it &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;links to this ion&lt;/scene&gt; , it becomes a homooligomer.<br /> MICU 1 is composed by differents domains : <br /> :- C helix region (or Coiled-coil domain) : this domain permits different interactions with other proteins (MCU/MICU2) and is required to assemble free Ca2+ with homohexamer.<br /> :- Polybasic region : it’s a domain which permits protein-protein interactions <br /> :- &lt;scene name='72/723172/Ef_domains/1'&gt;EF-hand domains&lt;/scene&gt; There are 2 of them. They are the most important domains in MICU1 protein because they contain the Ca2+ binding region and detect the concentration of calcium in the cell for activation of signalling. An EF-hand domain is a calcium sensor. An EF-hand domain is a helix loop helix structural domain that means that it is 2 alpha helices linked by a short loop region. Ca2+ ions are coordinated in this space thanks to ligands within the loop : in EF-1 residues concerning are Asp231, Asn233, Asp235, Glu237 and Glu242 and in EF-2 they are Asp421, Asp423, Asn425, Glu427 and Glu432. When Ca2+ binds itself to this domain, the protein changes its conformation to expose a domain that can interact with other proteins.<br /> <br /> == See Also ==<br /> <br /> :MCU : http://www.rcsb.org/pdb/gene/MCU<br /> :MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525544 2016-01-28T14:54:39Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> The protein has a rich &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt;. It has alpha helix, beta sheets and loops. <br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> In this figure,&lt;scene name='72/723172/Ef_domains/1'&gt;the EF domains&lt;/scene&gt; of the hexametric MICU 1 protein are shown in purple. <br /> Here is the &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;binding site of calcium&lt;/scene&gt; in the dimeric MICU 1 conformation. <br /> <br /> == See Also ==<br /> <br /> MCU : http://www.rcsb.org/pdb/gene/MCU<br /> MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525539 2016-01-28T14:50:42Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure==<br /> <br /> <br /> Ligands<br /> Ca2+<br /> Related<br /> MCU,MICU,MICU3<br /> Gene<br /> CBARA, Chromosome 10, 298865 pb (72,367,326 to 72,626,191)<br /> Activity<br /> Regulator of Ca 2+ uptakes in the mitochondria <br /> <br /> <br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> The protein has a rich &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt;. It has alpha helix, beta sheets and loops. <br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> In this figure,&lt;scene name='72/723172/Ef_domains/1'&gt;the EF domains&lt;/scene&gt; of the hexametric MICU 1 protein are shown in purple. <br /> Here is the &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;binding site of calcium&lt;/scene&gt; in the dimeric MICU 1 conformation. <br /> <br /> == See Also ==<br /> <br /> MCU : http://www.rcsb.org/pdb/gene/MCU<br /> MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525521 2016-01-28T14:34:46Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure)==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> MICU 1 is a regulator of Ca 2+ uptakes in mitochondria. To do this regulation, it can sense the concentration of calcium in the cytosol of the cell thanks to 2 EF-hand domains. It mainly acts on MCU which is a transmembrane uniporter channel located on the inner membrane of the mitochondria. To interact with MCU, MICU1 has to be linked to MICU2 (a paralogue protein). The entire complex of Ca2+ regulation is MICU1/MICU2/MCU/EMRE. EMRE is a protein which is required for the interaction of MCU with MICU1/MICU2.<br /> When it is not linked to Ca2+ it has an hexameric conformation. In this conformation MICU 1 silences the activity of MCU/EMRE and stop the Ca 2+ from entering the mitochondria by blocking the entrance of the channel. <br /> When there is a lot of calcium in the intermembrane so in the cytoplasm, Ca2+ binds to MICU1, then it changes its conformation, separates into multiple homooligomers that results in opens the “gate” in front of the MCU channel, letting the calcium enter into the matrix.<br /> <br /> <br /> == Disease ==<br /> If MICU1 gene is modified, Ca2+ can be load in higher concentration in the mitochondry resulting Myopathy with extrapyramidal signs (MPXPS). This is an autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> The protein has a rich &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt;. It has alpha helix, beta sheets and loops. <br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> In this figure,&lt;scene name='72/723172/Ef_domains/1'&gt;the EF domains&lt;/scene&gt; of the hexametric MICU 1 protein are shown in purple. <br /> Here is the &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;binding site of calcium&lt;/scene&gt; in the dimeric MICU 1 conformation. <br /> <br /> == See Also ==<br /> <br /> MCU : http://www.rcsb.org/pdb/gene/MCU<br /> MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525512 2016-01-28T14:29:59Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure)==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> The protein has a rich &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt;. It has alpha helix, beta sheets and loops. <br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> In this figure,&lt;scene name='72/723172/Ef_domains/1'&gt;the EF domains&lt;/scene&gt; of the hexametric MICU 1 protein are shown in purple. <br /> Here is the &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;binding site of calcium&lt;/scene&gt; in the dimeric MICU 1 conformation. <br /> <br /> == See Also ==<br /> <br /> MCU : http://www.rcsb.org/pdb/gene/MCU<br /> MICU 2 : http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525509 2016-01-28T14:29:19Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure)==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> The protein has a rich &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt;. It has alpha helix, beta sheets and loops. <br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> In this figure,&lt;scene name='72/723172/Ef_domains/1'&gt;the EF domains&lt;/scene&gt; of the hexametric MICU 1 protein are shown in purple. <br /> Here is the &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;binding site of calcium&lt;/scene&gt; in the dimeric MICU 1 conformation. <br /> <br /> == See Also ==<br /> <br /> http://www.rcsb.org/pdb/gene/MCU<br /> http://www.rcsb.org/pdb/gene/MICU2<br /> <br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525473 2016-01-28T14:08:22Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure)==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> The protein has a rich &lt;scene name='72/723172/Secondary_structure_of_micu_1/1'&gt;secondary structure&lt;/scene&gt;. It has alpha helix, beta sheets and loops. <br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> In this figure,&lt;scene name='72/723172/Ef_domains/1'&gt;the EF domains&lt;/scene&gt; of the hexametric MICU 1 protein are shown in purple. <br /> Here is the &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;binding site of calcium&lt;/scene&gt; in the dimeric MICU 1 conformation. <br /> <br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525461 2016-01-28T14:00:27Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure)==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> In this figure,&lt;scene name='72/723172/Ef_domains/1'&gt;the EF domains&lt;/scene&gt; of the hexametric MICU 1 protein are shown in purple. <br /> Here is the &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;binding site of calcium&lt;/scene&gt; in the dimeric MICU 1 conformation. <br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525448 2016-01-28T13:40:12Z

<p>Alexandra Helleux: </p> <hr /> <div>==Structure)==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;Here is the binding site of calcium in the dimer&lt;/scene&gt;<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525446 2016-01-28T13:37:46Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/3'&gt;Here is the binding site of calcium in the dimer&lt;/scene&gt;<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525443 2016-01-28T13:31:54Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/2'&gt;Here we can ses the binding site of the Calcium in the dimer&lt;/scene&gt;<br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525429 2016-01-28T13:10:40Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> &lt;scene name='72/723172/Protein_dimer_when_ca_is_bound/1'&gt;The structure of this is the following&lt;/scene&gt;<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525409 2016-01-28T12:42:12Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> &lt;Structure load='4NSD' size='150' frame='true' align='right' caption='Conformation when CA is bound' scene='Dimerization' /&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525408 2016-01-28T12:41:37Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> &lt;Structure load='4NSD' size='200' frame='true' align='right' caption='Conformation when CA is bound' scene='Dimerization' /&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525407 2016-01-28T12:40:59Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> &lt;Structure load='4NSD' size='350' frame='true' align='right' caption='Conformation when CA is bound' scene='Dimerization' /&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525406 2016-01-28T12:38:23Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSD' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2525405 2016-01-28T12:37:17Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> &lt;StructureSection load='4NSD' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2523302 2016-01-27T16:29:03Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2523301 2016-01-27T16:28:07Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> <br /> Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high.<br /> It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake.<br /> <br /> == Function ==<br /> <br /> Micu 1 is a regulator of Ca 2+ uptakes in mitochondria.<br /> It mainly acts on MCU when linked to MICU2 (paralogues).<br /> When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria.<br /> When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix.<br /> <br /> == Disease ==<br /> <br /> The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). <br /> But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> Ca 2+ binds to EF domain on MICU 1 and 2<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2523049 2016-01-26T18:45:10Z

<p>Alexandra Helleux: </p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='4NSC' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> This is a default text for your page '''Micu 1'''. Click above on '''edit this page''' to modify. Be careful with the &amp;lt; and &amp;gt; signs.<br /> You may include any references to papers as in: the use of JSmol in Proteopedia &lt;ref&gt;DOI 10.1002/ijch.201300024&lt;/ref&gt; or to the article describing Jmol &lt;ref&gt;PMID:21638687&lt;/ref&gt; to the rescue.<br /> <br /> == Function ==<br /> <br /> == Disease ==<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux https://proteopedia.org/index.php?title=Calcium_uptake_protein_1&diff=2523047 2016-01-26T18:43:14Z

<p>Alexandra Helleux: New page: ==Your Heading Here (maybe something like &#039;Structure&#039;)== &lt;StructureSection load=&#039;1stp&#039; size=&#039;340&#039; side=&#039;right&#039; caption=&#039;Caption for this structure&#039; scene=&#039;&#039;&gt; This is a default text for you...</p> <hr /> <div>==Your Heading Here (maybe something like 'Structure')==<br /> &lt;StructureSection load='1stp' size='340' side='right' caption='Caption for this structure' scene=''&gt;<br /> This is a default text for your page '''Micu 1'''. Click above on '''edit this page''' to modify. Be careful with the &amp;lt; and &amp;gt; signs.<br /> You may include any references to papers as in: the use of JSmol in Proteopedia &lt;ref&gt;DOI 10.1002/ijch.201300024&lt;/ref&gt; or to the article describing Jmol &lt;ref&gt;PMID:21638687&lt;/ref&gt; to the rescue.<br /> <br /> == Function ==<br /> <br /> == Disease ==<br /> <br /> == Relevance ==<br /> <br /> == Structural highlights ==<br /> <br /> This is a sample scene created with SAT to &lt;scene name=&quot;/12/3456/Sample/1&quot;&gt;color&lt;/scene&gt; by Group, and another to make &lt;scene name=&quot;/12/3456/Sample/2&quot;&gt;a transparent representation&lt;/scene&gt; of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.<br /> <br /> &lt;/StructureSection&gt;<br /> == References ==<br /> &lt;references/&gt;</div>

Alexandra Helleux