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]


  1. Anonymous (2015), Complement C1 inhibitor protein (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. Markovic SN, Inwards DJ, Frigas EA, Phyliky RP (January 2000). "Acquired C1 esterase inhibitor deficiency". Ann. Intern. Med. 132 (2): 144–50. PMID 10644276[e]
  3. Cicardi M, Zingale LC, Pappalardo E, Folcioni A, Agostoni A (July 2003). "Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies". Medicine (Baltimore) 82 (4): 274–81. DOI:10.1097/ PMID 12861105. Research Blogging.