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Molecular Biology Commons

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Developmental Biology

University of Massachusetts Medical School

Peter Jones Lab Publications

2009

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Full-Text Articles in Molecular Biology

Fshd Region Gene 1 (Frg1) Is Crucial For Angiogenesis Linking Frg1 To Facioscapulohumeral Muscular Dystrophy-Associated Vasculopathy, Ryan Wuebbles, Meredith L. Hanel, Peter L. Jones May 2009

Fshd Region Gene 1 (Frg1) Is Crucial For Angiogenesis Linking Frg1 To Facioscapulohumeral Muscular Dystrophy-Associated Vasculopathy, Ryan Wuebbles, Meredith L. Hanel, Peter L. Jones

Peter Jones Lab Publications

The genetic lesion that is diagnostic for facioscapulohumeral muscular dystrophy (FSHD) results in an epigenetic misregulation of gene expression, which ultimately leads to the disease pathology. FRG1 (FSHD region gene 1) is a leading candidate for a gene whose misexpression might lead to FSHD. Because FSHD pathology is most prominent in the musculature, most research and therapy efforts focus on muscle cells. Previously, using Xenopus development as a model, we showed that altering frg1 expression levels systemically leads to aberrant muscle development, illustrating the potential for aberrant FRG1 levels to disrupt the musculature. However, 50-75% of FSHD patients also exhibit ...