4lmf

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Template:STRUCTURE 4lmf

Contents

C1s CUB1-EGF-CUB2

Template:ABSTRACT PUBMED 23922389

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

4lmf is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Venkatraman Girija U, Gingras AR, Marshall JE, Panchal R, Sheikh MA, Gal P, Schwaeble WJ, Mitchell DA, Moody PC, Wallis R. Structural basis of the C1q/C1s interaction and its central role in assembly of the C1 complex of complement activation. Proc Natl Acad Sci U S A. 2013 Aug 20;110(34):13916-20. doi:, 10.1073/pnas.1311113110. Epub 2013 Aug 6. PMID:23922389 doi:10.1073/pnas.1311113110

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