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Facioscapulohumeral muscular dystrophy

  • RESEARCH ARTICLE
    p53-independent DUX4 pathology in cell and animal models of facioscapulohumeral muscular dystrophy
    Darko Bosnakovski, Micah D. Gearhart, Erik A. Toso, Olivia O. Recht, Anja Cucak, Abhinav K. Jain, Michelle C. Barton, Michael Kyba
    Disease Models & Mechanisms 2017 10: 1211-1216; doi: 10.1242/dmm.030064

    Summary: DUX4 is thought to mediate cytopathology through p53. Here, DUX4 is shown to kill primary myoblasts and promote pathological phenotypes in the iDUX4[2.7] mouse model on the p53-null background, calling into question this notion.

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