4j1y
From Proteopedia
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==About this Structure== | ==About this Structure== | ||
[[4j1y]] is a 2 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=4J1Y OCA]. | [[4j1y]] is a 2 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=4J1Y OCA]. | ||
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| + | ==Reference== | ||
| + | <ref group="xtra">PMID:023592783</ref><references group="xtra"/><references/> | ||
[[Category: Complement subcomponent C1s]] | [[Category: Complement subcomponent C1s]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
Revision as of 08:44, 19 June 2013
Contents |
The X-ray crystal structure of human complement protease C1s zymogen
Template:ABSTRACT PUBMED 23592783
Disease
[C1S_HUMAN] Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:613783]. 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
[C1S_HUMAN] C1s B chain is a serine protease that combines with C1q and C1r to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.
About this Structure
4j1y is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Perry AJ, Wijeyewickrema LC, Wilmann PG, Gunzburg MJ, D'Andrea L, Irving JA, Pang SS, Duncan RC, Wilce JA, Whisstock JC, Pike RN. A molecular switch governs the interaction between human complement protease C1s and its substrate, complement C4. J Biol Chem. 2013 Apr 16. PMID:23592783 doi:10.1074/jbc.M113.464545
