C1 inhibitor, human

C1 inhibitor, human is a purified plasma-derived protein that functions as a serine protease inhibitor (serpin) and is used to treat and prevent hereditary angioedema (HAE), a rare genetic disorder characterized by recurrent episodes of severe swelling in various parts of the body. The drug works by replacing deficient or dysfunctional endogenous C1-inhibitor in patients with HAE. Mechanistically, it inhibits several key proteins in the complement and contact system pathways—including C1s, kallikrein, factor XIIa, and XIa—thereby preventing excessive bradykinin production and subsequent increases in vascular permeability that lead to angioedema attacks. It is administered intravenously or subcutaneously depending on the product formulation. Brand names include Berinert (for acute attacks), Cinryze (for prophylaxis), and Haegarda (for prophylaxis). The primary indication is hereditary angioedema[3][5][6][8].

Brand names

BerinertCinryzeHaegarda

Other names

c1 inhibitor, humanc1 esterase inhibitor, humancomplement component 1 esterase inhibitor, human