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- Sacha Zeerleder.
- Department of Immunopathology, Sanquin Research and Landsteiner Laboratory of the AMC, Amsterdam, The Netherlands. s.zeerleder@sanquin.nl
- Semin. Thromb. Hemost. 2011 Jun 1;37(4):362-74.
AbstractC1-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.© Thieme Medical Publishers.
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