C1-esterase inhibitor

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 01.08.2022

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Glycoprotein (105 KDa), which is predominantly formed in hepatocytes, consists of 487 amino acids and regulates the activation of complement factor C1.

General information
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The human C1 esterase inhibitor gene is located on the eleventh chromosome (11q11-q13.1). Deficiency of the C1 esterase inhibitor leads to activation of the complement system, which in turn leads to low plasma concentrations of complement factor C2. It is currently believed that cleaved C2 increases the permeability of vascular walls. Furthermore, it has long been hypothesized that inhibitor deficiency results in increased kallikrein and, consequently, increased bradykinin, which causes the subcutaneous swelling seen in typeI hereditary angioedema. Elevated levels of bradykinin have been noted in acute swellings.

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The standard value in the blood is about 0.25-0.45 g/l.

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  1. Zuraw BL (2006) Novel therapies for hereditary angioedema. Immunol Allergy Clin North Am 26: 691-708
  2. Levy JH et al (2006) The therapeutic potential of a kallikrein inhibitor for treating hereditary angioedema. Expert Opinion Investig Drugs 15: 1077-1090

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Angioedema hereditary 1;


Last updated on: 01.08.2022