Structure, function and molecular genetics of human and murine C1r.

Abstract:

:C1r, the enzyme responsible for intrinsic activation of the C1 complex of complement, is a modular serine protease featuring an overall structural organization homologous to those of C1s and the mannan-binding lectin-associated serine proteases (MASPs). This review will initially summarize current information on the structure and function of C1r, with particular emphasis on the three-dimensional structure of its catalytic domain, which provides new insights into the activation mechanism of C1. The second part of this review will focus on recent discoveries dealing with a truncated, C1r-related protein, and the occurrence in the mouse of two isoforms, C1rA and C1rB, exhibiting tissue-specific expression patterns.

journal_name

Immunobiology

journal_title

Immunobiology

authors

Arlaud GJ,Gaboriaud C,Garnier G,Circolo A,Thielens NM,Budayova-Spano M,Fontecilla-Camps JC,Volanakis JE

doi

10.1078/0171-2985-00139

subject

Has Abstract

pub_date

2002-09-01 00:00:00

pages

365-82

issue

4-5

eissn

0171-2985

issn

1878-3279

pii

S0171-2985(04)70088-1

journal_volume

205

pub_type

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