2jg8: Difference between revisions

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<StructureSection load='2jg8' size='340' side='right'caption='[[2jg8]], [[Resolution|resolution]] 2.05&Aring;' scene=''>
<StructureSection load='2jg8' size='340' side='right'caption='[[2jg8]], [[Resolution|resolution]] 2.05&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[2jg8]] is a 6 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2JG8 OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=2JG8 FirstGlance]. <br>
<table><tr><td colspan='2'>[[2jg8]] is a 6 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=2JG8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2JG8 FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SEP:PHOSPHOSERINE'>SEP</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]] 2.05&#8491;</td></tr>
<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2jg9|2jg9]], [[1pk6|1pk6]]</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SEP:PHOSPHOSERINE'>SEP</scene></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=2jg8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2jg8 OCA], [http://pdbe.org/2jg8 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2jg8 RCSB], [http://www.ebi.ac.uk/pdbsum/2jg8 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2jg8 ProSAT]</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=2jg8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2jg8 OCA], [https://pdbe.org/2jg8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2jg8 RCSB], [https://www.ebi.ac.uk/pdbsum/2jg8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2jg8 ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/C1QA_HUMAN C1QA_HUMAN]] Defects in C1QA are a cause of complement component C1q deficiency (C1QD) [MIM:[http://omim.org/entry/613652 613652]]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis. [[http://www.uniprot.org/uniprot/C1QC_HUMAN C1QC_HUMAN]] Defects in C1QC are a cause of complement component C1q deficiency (C1QD) [MIM:[http://omim.org/entry/613652 613652]]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis.<ref>PMID:8630118</ref>  [[http://www.uniprot.org/uniprot/C1QB_HUMAN C1QB_HUMAN]] Defects in C1QB are a cause of complement component C1q deficiency (C1QD) [MIM:[http://omim.org/entry/613652 613652]]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis.<ref>PMID:9476130</ref> 
[https://www.uniprot.org/uniprot/C1QA_HUMAN C1QA_HUMAN] Defects in C1QA are a cause of complement component C1q deficiency (C1QD) [MIM:[https://omim.org/entry/613652 613652]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis.
== Function ==
== Function ==
[[http://www.uniprot.org/uniprot/C1QA_HUMAN C1QA_HUMAN]] C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes. [[http://www.uniprot.org/uniprot/C1QC_HUMAN C1QC_HUMAN]] C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes. [[http://www.uniprot.org/uniprot/C1QB_HUMAN C1QB_HUMAN]] C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes.  
[https://www.uniprot.org/uniprot/C1QA_HUMAN C1QA_HUMAN] C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes.
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
[[Category: Arlaud, G J]]
[[Category: Arlaud GJ]]
[[Category: Darnault, C]]
[[Category: Darnault C]]
[[Category: Frachet, P]]
[[Category: Frachet P]]
[[Category: Gaboriaud, C]]
[[Category: Gaboriaud C]]
[[Category: Garlatti, V]]
[[Category: Garlatti V]]
[[Category: Ghebrehiwet, B]]
[[Category: Ghebrehiwet B]]
[[Category: Paidassi, H]]
[[Category: Paidassi H]]
[[Category: Tacnet-Delorme, P]]
[[Category: Tacnet-Delorme P]]
[[Category: Apopotosis]]
[[Category: Cell surface molecule]]
[[Category: Collagen]]
[[Category: Complement]]
[[Category: Complement pathway]]
[[Category: Disease mutation]]
[[Category: Glycoprotein]]
[[Category: Hydroxylation]]
[[Category: Immune response]]
[[Category: Immune system]]
[[Category: Innate immunity]]
[[Category: Phagocytosis]]
[[Category: Polymorphism]]
[[Category: Pyrrolidone carboxylic acid]]
[[Category: Tolerance]]