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C1 Inhibitor and Hereditary Angioneurotic Edema

C1 Inhibitor and Hereditary Angioneurotic Edema CI inhibitor (CI INH) is a protease inhibitor involved in the regulation of several proteolytic systems in plasma, including the complement, coagu­ lation, fbrinolytic, i physiologic role of this protein is best demonstrated by the dramatic symptoms and biochemical abnormalities in individuals with either hered­ itary Cl INH deficiency (hereditary angioneurotic edema-HANE), or the syndrome of acquired CI INH defciency. i learned in the past several years about the structure, genetics, mechanism of action, and inhibitory spectrum of C1 INH, numerous questions in each of these areas remain unresolved. For example, the pathophysiology of the generation of symptoms in HANE remains inadequately defined . CI INH is clearly the only plasma protease inhibitor that regulates Cl acti­ vation, and it is the major inhibitor of the enzymes involved in activation of the contact system. As might therefore be predicted, evidence exists for both complement and contact system activation in HANE, but the major mediator (or mediators) has not been fully characterized. Similarly, only fragmentary information is currently available regarding the specific molecular genetic defects in the Cl INH gene that lead to deficiency, although the recent cloning of the gene should rapidly yield information in this regard. A http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Annual Review of Immunology Annual Reviews

C1 Inhibitor and Hereditary Angioneurotic Edema

Annual Review of Immunology , Volume 6 (1) – Apr 1, 1988

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Publisher
Annual Reviews
Copyright
Copyright 1988 Annual Reviews. All rights reserved
Subject
Review Articles
ISSN
0732-0582
eISSN
1545-3278
DOI
10.1146/annurev.iy.06.040188.003115
pmid
3289579
Publisher site
See Article on Publisher Site

Abstract

CI inhibitor (CI INH) is a protease inhibitor involved in the regulation of several proteolytic systems in plasma, including the complement, coagu­ lation, fbrinolytic, i physiologic role of this protein is best demonstrated by the dramatic symptoms and biochemical abnormalities in individuals with either hered­ itary Cl INH deficiency (hereditary angioneurotic edema-HANE), or the syndrome of acquired CI INH defciency. i learned in the past several years about the structure, genetics, mechanism of action, and inhibitory spectrum of C1 INH, numerous questions in each of these areas remain unresolved. For example, the pathophysiology of the generation of symptoms in HANE remains inadequately defined . CI INH is clearly the only plasma protease inhibitor that regulates Cl acti­ vation, and it is the major inhibitor of the enzymes involved in activation of the contact system. As might therefore be predicted, evidence exists for both complement and contact system activation in HANE, but the major mediator (or mediators) has not been fully characterized. Similarly, only fragmentary information is currently available regarding the specific molecular genetic defects in the Cl INH gene that lead to deficiency, although the recent cloning of the gene should rapidly yield information in this regard. A

Journal

Annual Review of ImmunologyAnnual Reviews

Published: Apr 1, 1988

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