Expert Reviews in Molecular Medicine: http://www.expertreviews.org/
DOI: 10.1017/S1462399406000184; 11 December 2006
Dion Kaiserman, James C. Whisstock and Phillip I. Bird (2006) Mechanisms of serpin dysfunction in disease. Expert Rev. Mol. Med. Vol. 8, Issue 31, DOI: 10.1017/S1462399406000184

Mechanisms of serpin dysfunction in disease

Dion Kaiserman ^{a1 c1}, James C. Whisstock ^a1 and Phillip I. Bird ^a1

^a1 Department of Biochemistry and Molecular Biology, Monash University, Clayton, VIC 3800, Australia.

^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

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 a₁-antitrypsin Pittsburgh mutation causes a change in target proteinase; the a₁-antitrypsin Z mutation and neuroserpin, polymerisation of which lead to cellular cytotoxicity; and a loss of maspin expression resulting in cancer.

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