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