1elv

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Template:STRUCTURE 1elv

Contents 1 CRYSTAL STRUCTURE OF THE CATALYTIC DOMAIN OF HUMAN COMPLEMENT C1S PROTEASE 2 Disease 3 Function 4 About this Structure 5 Reference

CRYSTAL STRUCTURE OF THE CATALYTIC DOMAIN OF HUMAN COMPLEMENT C1S PROTEASE

Template:ABSTRACT PUBMED 10775260

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

1elv is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

• Gaboriaud C, Rossi V, Bally I, Arlaud GJ, Fontecilla-Camps JC. Crystal structure of the catalytic domain of human complement c1s: a serine protease with a handle. EMBO J. 2000 Apr 17;19(8):1755-65. PMID:10775260 doi:http://dx.doi.org/10.1093/emboj/19.8.1755