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Semin Thromb Hemost 2011; 37(4): 362-374
DOI: 10.1055/s-0031-1276585
© Thieme Medical Publishers

C1-Inhibitor: More Than a Serine Protease Inhibitor

Sacha Zeerleder1
  • 1Department of Immunopathology, Sanquin Research and Landsteiner Laboratory of the AMC, Amsterdam, The Netherlands and Department of Hematology, Academic Medical Center, University of Amsterdam, The Netherlands
Further Information

Publication History

Publication Date:
30 July 2011 (online)

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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.

KEYWORDS

C1-inhibitor - hereditary angioedema - serpin - contact phase - complement - sepsis

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Sacha ZeerlederM.D. Ph.D. 

Department of Immunopathology, Sanquin Research, Plesmanlaan 125

1066 CX Amsterdam, The Netherlands

Email: s.zeerleder@sanquin.nl