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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 assumed that the cleaved C2 increases the permeability of the vessel walls. Furthermore, it has long been assumed that the inhibitor deficiency leads to increased kallikrein and consecutively to increased bradykinin, which causes subcutaneous swelling in hereditary angioedema. Elevated bradykinin levels have been found in acute swelling.
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LiteratureThis section has been translated automatically.
- Zuraw BL (2006) Novel therapies for hereditary angioedema. Immunol Allergy Clin North Am 26: 691-708
- Levy JH et al (2006) The therapeutic potential of a kallikrein inhibitor for treating hereditary angioedema. Expert Opinion Investig Drugs 15: 1077-1090