Complement C1 Inhibitor Protein

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Complement C1 Inhibitor Protein

An endogenous serine protease inhibitor (SERPINS). It is a 105-kDA plasma glycoprotein, encoded by C1NH gene and produced primarily by the liver and monocytes. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from hereditary ANGIONEUROTIC EDEMA.