CJO - Abstract - Mechanisms of serpin dysfunction in disease

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Expert Reviews in Molecular Medicine (2006), 8 : 1-19 Cambridge University Press
Copyright © 2006 Cambridge University Press
doi:10.1017/S1462399406000184 (About doi)
Published online by Cambridge University Press 11 Dec 2006
 
Expert Reviews in Molecular Medicine (2006), 8:31:1-19 Cambridge University Press
Copyright © 2006 Cambridge University Press
doi:10.1017/S1462399406000184

Review Article

Mechanisms of serpin dysfunction in disease

Dion Kaiserman a1c1 , James C. Whisstock a1 and Phillip I. Bird a1
a1 Department of Biochemistry and Molecular Biology, Monash University, Clayton, VIC 3800, Australia.

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Abstract

The serpin superfamily encompasses hundreds of proteins, spread across all kingdoms of life, linked by a common tertiary fold. This review focuses on five diseases caused by serpin dysfunction: variants of antithrombin III lose their ability to interact with heparin; the α1-antitrypsin Pittsburgh mutation causes a change in target proteinase; the α1-antitrypsin Z mutation and neuroserpin, polymerisation of which lead to cellular cytotoxicity; and a loss of maspin expression resulting in cancer.


Correspondence:
c1 Corresponding author: Dion Kaiserman, Building 13B, Department of Biochemistry and Molecular Biology, Monash University, Clayton, VIC 3800, Australia. Tel: +61 03 99055214; Fax: +61 03 99053726; E-mail: dion.kaiserman@med.monash.edu.au

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