Overexpression of facioscapulohumeral muscular dystrophy region gene 1 causes primary defects in myogenic stem cells.

作者: A. Xynos , M. V. Neguembor , R. Caccia , D. Licastro , A. Nonis

DOI: 10.1242/JCS.121533

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摘要: Summary Overexpression of facioscapulohumeral muscular dystrophy region gene 1 (FRG1) in mice, frogs and worms leads to vascular abnormalities. Nevertheless, the mechanism that follows FRG1 overexpression finally defects is currently unknown. Here, we show earliest phenotype displayed by mice overexpressing a postnatal muscle-growth defect. Long before development dystrophy, also exhibit muscle regeneration impairment. Ex vivo experiments revealed causes myogenic stem cell activation proliferative, clonogenic differentiation defects. A comparative expression profiling muscles from young pre-dystrophic wild-type identified differentially expressed genes several categories networks could explain emerging tissue Overall, our study provides new insights into pathways regulated suggests contribute pathology mice.

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