Complement C1 inhibitor protein

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In medicine and immunology, complement C1 inhibitor protein (C1 esterase inhibitor or C1 INH protein) is 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".^[1]

Angioedema due to deficiency of complement C1 inhibitor protein may also be acquired.^[2][3]

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