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  • Title: Mechanisms of serpin dysfunction in disease.
    Author: Kaiserman D, Whisstock JC, Bird PI.
    Journal: Expert Rev Mol Med; 2006 Dec 11; 8(31):1-19. PubMed ID: 17156576.
    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 alpha1-antitrypsin Pittsburgh mutation causes a change in target proteinase; the alpha1-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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