1nzi
From Proteopedia
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{{STRUCTURE_1nzi| PDB=1nzi | SCENE= }} | {{STRUCTURE_1nzi| PDB=1nzi | SCENE= }} | ||
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===Crystal Structure of the CUB1-EGF Interaction Domain of Complement Protease C1s=== | ===Crystal Structure of the CUB1-EGF Interaction Domain of Complement Protease C1s=== | ||
| + | {{ABSTRACT_PUBMED_12788922}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/C1S_HUMAN C1S_HUMAN]] Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:[http://omim.org/entry/613783 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. | ||
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| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/C1S_HUMAN 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== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:012788922</ref><references group="xtra"/> | + | <ref group="xtra">PMID:012788922</ref><references group="xtra"/><references/> |
[[Category: Complement subcomponent C1s]] | [[Category: Complement subcomponent C1s]] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
Revision as of 23:35, 24 March 2013
Contents |
Crystal Structure of the CUB1-EGF Interaction Domain of Complement Protease C1s
Template:ABSTRACT PUBMED 12788922
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
1nzi is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Gregory LA, Thielens NM, Arlaud GJ, Fontecilla-Camps JC, Gaboriaud C. X-ray structure of the Ca2+-binding interaction domain of C1s. Insights into the assembly of the C1 complex of complement. J Biol Chem. 2003 Aug 22;278(34):32157-64. Epub 2003 Jun 4. PMID:12788922 doi:http://dx.doi.org/10.1074/jbc.M305175200
