title: C1-inhibitor: more than a serine protease inhibitor.
abstract: C1-inhibitor (C1-inh) is a crucial regulator of the activation of plasmatic cascade systems involved in inflammation contributing to the homeostasis in the generation of proinflammatory mediators. The importance of C1-inh is illustrated by patients with hereditary angioedema where decreased levels of C1-inh lead to an uncontrolled generation of vasoactive peptides resulting in potential life-threatening subcutaneous edema. Recent publications, however, suggest that the anti-inflammatory properties of C1-inh do not strictly depend on its capacity to regulate the complement and contact phase system. This review summarizes the biochemical characteristics of C1-inh and its role in the regulation of plasmatic cascade systems as well as the role of the nonserpin domain.
authors.affiliation: Department of Immunopathology, Sanquin Research and Landsteiner Laboratory of the AMC, Amsterdam, The Netherlands. s.zeerleder@sanquin.nl
authors.forename: Sacha
authors.initials: S
authors.lastname: Zeerleder
mesh_terms: D000818:Animals; D050718:Complement C1 Inhibitor Protein / Q000737:chemistry / Q000502:physiology*; D005342:Fibrinolysis / Q000502:physiology; D006801:Humans
chemical_list: D050718:Complement C1 Inhibitor Protein