Complement 1 Inhibitor (C1INH)

SERPING1; C1IN; C1-INH; C1NH; HAE1; HAE2; Serpin Peptidase Inhibitor Clade G Member 1; Angioedema Hereditary; C1 Inhibitor; C1 Esterase Inhibitor

SPN	W513
HGNC	1228
MGI	894696
PubMed	22882460
RGD	735225
UniProt	P05155

C1-INH is a critically important protein that controls activation of multiple plasma mediator pathways. This protein, a member of the serine protease inhibitor (serpin) group, originally was described as an inhibitor of C1. It binds stoichiometrically to the active sites on both C1r and C1s to form a complex C1-INH-C1r-C1s-C1-INH and thus inhibits activated C1. In addition, C1-INH has been reported to remove the intact C1qrs complex from an activating surface and to inhibit autoactivation of C1. C1-INH is a known inhibitor of kinin generating (kallikrein), fibrinolytic (plasmin), and contact activation (intrinsic) pathway of the coagulation cascade. Recently, it has been shown to be an inhibitor of the mannan-binding lectin pathway of complement activation, inhibiting mannan-binding lectin-associated serine proteases (MASPs) in that pathway.

 

Reference

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"N-linked glycosylation at Asn3 and the positively charged residues within the amino-terminal domain of the c1 inhibitor are required for interaction of the C1 Inhibitor with Salmonella enterica serovar typhimurium lipopolysaccharide and lipid A."
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