Target intelligence / Profile preview

Serpin Family G Member 1 (C1-INH)

Target
C1-INH
Molecular classification
Serpin, Serine Protease Inhibitor
01

Overview

Serpin Family G Member 1 (C1-INH) is a serine protease inhibitor that plays a critical regulatory role in the complement system and contact system. It inhibits activated components of the classical complement pathway (C1r and C1s) and also controls plasma kallikrein and activated factor XII (factor XIIa), which are involved in bradykinin production. Deficiency or dysfunction of C1-INH leads to hereditary angioedema.

Other names
C1INHIC1_HUMANPlasma protease C1 inhibitorSerine/cysteine proteinase inhibitor clade G member 1
02

Mechanism of action

Irreversible inhibition of target proteases (C1r, C1s, plasma kallikrein, Factor XIIa) through stable complex formation via the reactive center loop (RCL).

03

Biological functions

Complement regulationContact system regulationInhibition of C1r and C1sInhibition of plasma kallikreinInhibition of Factor XIIaRegulation of bradykinin production
04

Disease associations

Hereditary AngioedemaAutoimmune DiseasesAge-related Macular DegenerationInflammation
05

Biomarkers

C1-INH levels (quantitative deficiency)C1-INH activity (qualitative dysfunction)

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