Description: |
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 ANGIOEDEMA. |