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]

References

  1. ↑ Anonymous, (2009) 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.
  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:10.1097/01.md.0000085055.63483.09. PMID 12861105.
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