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==Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthes==
==Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthes==
<StructureSection load='4ped' size='340' side='right' caption='[[4ped]], [[Resolution|resolution]] 1.64&Aring;' scene=''>
<StructureSection load='4ped' size='340' side='right'caption='[[4ped]], [[Resolution|resolution]] 1.64&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[4ped]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4PED OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4PED FirstGlance]. <br>
<table><tr><td colspan='2'>[[4ped]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4PED OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4PED FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.64&#8491;</td></tr>
<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4ped FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ped OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4ped RCSB], [http://www.ebi.ac.uk/pdbsum/4ped PDBsum]</span></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4ped FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ped OCA], [https://pdbe.org/4ped PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4ped RCSB], [https://www.ebi.ac.uk/pdbsum/4ped PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4ped ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/ADCK3_HUMAN ADCK3_HUMAN]] Autosomal recessive ataxia due to ubiquinone deficiency. The disease is caused by mutations affecting the gene represented in this entry.  
[https://www.uniprot.org/uniprot/COQ8A_HUMAN COQ8A_HUMAN] Autosomal recessive ataxia due to ubiquinone deficiency. The disease is caused by variants affecting the gene represented in this entry.
== Function ==
== Function ==
[[http://www.uniprot.org/uniprot/ADCK3_HUMAN ADCK3_HUMAN]] Putative protein kinase involved in the biosynthesis of coenzyme Q since it is able to rescue partially coenzyme Q6 biosynthesis of yeast COQ8 mutants. May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.<ref>PMID:21296186</ref>
[https://www.uniprot.org/uniprot/COQ8A_HUMAN COQ8A_HUMAN] Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:25498144, PubMed:21296186, PubMed:25540914, PubMed:27499294). Its substrate specificity is unclear: does not show any protein kinase activity (PubMed:25498144, PubMed:27499294). Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates (PubMed:25498144, PubMed:27499294). Shows an unusual selectivity for binding ADP over ATP (PubMed:25498144).<ref>PMID:25498144</ref> <ref>PMID:27499294</ref> <ref>PMID:21296186</ref> <ref>PMID:25540914</ref>
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
</div>
<div class="pdbe-citations 4ped" style="background-color:#fffaf0;"></div>
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Barber, G E]]
[[Category: Homo sapiens]]
[[Category: Bingman, C A]]
[[Category: Large Structures]]
[[Category: Coon, J J]]
[[Category: Barber GE]]
[[Category: Floyd, B J]]
[[Category: Bingman CA]]
[[Category: Jochem, A]]
[[Category: Coon JJ]]
[[Category: Johnson, I E]]
[[Category: Floyd BJ]]
[[Category: Joshi, S]]
[[Category: Jochem A]]
[[Category: Kannan, N]]
[[Category: Johnson IE]]
[[Category: Lee, D]]
[[Category: Joshi S]]
[[Category: Li, S]]
[[Category: Kannan N]]
[[Category: MPP, Mitochondrial Protein Partnership]]
[[Category: Lee D]]
[[Category: Oruganty, O]]
[[Category: Li S]]
[[Category: Pagliarini, D J]]
[[Category: Oruganty O]]
[[Category: Reidenbach, A G]]
[[Category: Pagliarini DJ]]
[[Category: Saunders, J M]]
[[Category: Reidenbach AG]]
[[Category: Smith, R]]
[[Category: Saunders JM]]
[[Category: Stefely, J A]]
[[Category: Smith R]]
[[Category: Ulbrich, A]]
[[Category: Stefely JA]]
[[Category: Wrobel, R L]]
[[Category: Ulbrich A]]
[[Category: Coenzyme q biosynthesis]]
[[Category: Wrobel RL]]
[[Category: Membrane associated]]
[[Category: Mitochondrial]]
[[Category: Mitochondrial protein partnership]]
[[Category: Mpp]]
[[Category: Protein kinase-like]]
[[Category: Psi-biology]]
[[Category: Structural genomic]]
[[Category: Transferase]]