DEFINING THE BIOLOGICAL FUNCTION OF C1 INHIBITOR IN HEREDITARY ANGIOEDEMA

 
 
 
 

DEFINING THE BIOLOGICAL FUNCTION OF C1 INHIBITOR IN HEREDITARY ANGIOEDEMA

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Title: DEFINING THE BIOLOGICAL FUNCTION OF C1 INHIBITOR IN HEREDITARY ANGIOEDEMA
Author: HAN, EUN DUK
Description: C1 inhibitor (C1 INH) is a member of the serpin family (serine protease inhibitors) that plays an essential role in the regulation of classical complement activation by mainly inhibiting C1s and C1r. C1 INH is also the inhibitor of contact system components such as coagulation factors XIa and XIIa, plasmin, kallikrein, and tissue plasminogen activator. Patients with hereditary angioedema (HAE), an autosomal dominant disease, are found to have drastically reduced levels of functional C1 INH in their plasma. Reduced levels of C1 INH may lead to unregulated activation of the classical complement and contact cascades. This can potentially lead to development of edema of subcutaneous tissues, bowel walls and upper airways. The symptoms are unpredictable in frequency and severity. However, recurrent angioedema of the larynx can be life threatening. As many as 30% of patients died of asphyxiation before modern prophylactic therapy was available. Thus, understanding the mediation of angioedema and applying appropriate treatment have been the goals of many studies. Currently, the structure, genetics, mechanism of action, and inhibitory spectrum of C1 INH is well established. However, the precise biological function of C1 INH is ncompletely understood. Therefore, our laboratory has attempted to generate mice deficient in C1 INH to utilize these animals to define the biological role of C1 INH, to determine the potential mediator of symptoms in HAE, and to find a better therapeutic agent to treat HAE patients.
Permanent Link: http://rave.ohiolink.edu/etdc/view?acc_num=ucin1022759212
http://hdl.handle.net/2374.OX/12167
Date: 2002

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