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Full text of this paper is not available in the ETD Center. Copies may be available for inter-library loan from University of Cincinnati or may be available for purchase from Proquest/UMI

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DEFINING THE BIOLOGICAL FUNCTION OF C1 INHIBITOR IN HEREDITARY ANGIOEDEMA

HAN, EUN DUK
http://rave.ohiolink.edu/etdc/view?acc_num=ucin1022759212

Abstract Details

2002, PhD, University of Cincinnati, Medicine : Molecular and Developmental Biology.
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.
Dr. Jay Degen (Advisor)
1 p.
C1 INH; HAE; complement; contact system; vascular permeability

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Citations

  • HAN, E. D. (2002). DEFINING THE BIOLOGICAL FUNCTION OF C1 INHIBITOR IN HEREDITARY ANGIOEDEMA [Doctoral dissertation, University of Cincinnati]. OhioLINK Electronic Theses and Dissertations Center. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1022759212

    APA Style (7th edition)

  • HAN, EUN. DEFINING THE BIOLOGICAL FUNCTION OF C1 INHIBITOR IN HEREDITARY ANGIOEDEMA. 2002. University of Cincinnati, Doctoral dissertation. OhioLINK Electronic Theses and Dissertations Center, http://rave.ohiolink.edu/etdc/view?acc_num=ucin1022759212.

    MLA Style (8th edition)

  • HAN, EUN. "DEFINING THE BIOLOGICAL FUNCTION OF C1 INHIBITOR IN HEREDITARY ANGIOEDEMA." Doctoral dissertation, University of Cincinnati, 2002. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1022759212

    Chicago Manual of Style (17th edition)

ucin1022759212
© 2002, all rights reserved.
This open access ETD is published by University of Cincinnati and OhioLINK.