C1-inhibitor: more than a serine protease inhibitor

Semin Thromb Hemost. 2011 Jun;37(4):362-74. doi: 10.1055/s-0031-1276585. Epub 2011 Jul 30.

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.

Publication types

  • Review

MeSH terms

  • Animals
  • Complement C1 Inhibitor Protein / chemistry
  • Complement C1 Inhibitor Protein / physiology*
  • Fibrinolysis / physiology
  • Humans

Substances

  • Complement C1 Inhibitor Protein