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COL4A1

  • RESEARCH ARTICLE
    Use of sodium 4-phenylbutyrate to define therapeutic parameters for reducing intracerebral hemorrhage and myopathy in Col4a1 mutant mice
    Genki Hayashi, Cassandre Labelle-Dumais, Douglas B. Gould
    Disease Models & Mechanisms 2018 11: dmm034157 doi: 10.1242/dmm.034157 Published 4 July 2018

    Summary: COL4A1 mutations cause a multisystem disorder by impairing secretion of COL4A1/A2 heterotrimers. We define parameters for reducing stroke and myopathy in Col4a1 mutant mice by pharmacologically promoting heterotrimer secretion.

  • RESEARCH ARTICLE
    Genetic dissection of anterior segment dysgenesis caused by a Col4a1 mutation in mouse
    Mao Mao, Márton Kiss, Yvonne Ou, Douglas B. Gould
    Disease Models & Mechanisms 2017 10: 475-485; doi: 10.1242/dmm.027888

    Summary: Key pathogenic events in anterior segment dysgenesis, a congenital ocular disease with complex etiology, are recapitulated in a mouse model of Col4a1-related ASD.

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