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• W2899154927 abstract "Abstract Congenital muscular dystrophy with laminin α2 chain-deficiency (LAMA2-CMD) is a severe muscle disorder with complex underlying pathogenesis. We have previously employed profiling techniques to elucidate molecular patterns and demonstrated significant metabolic impairment in skeletal muscle from LAMA2-CMD patients and mouse models. Thus, we hypothesize that skeletal muscle metabolism may be a promising pharmacological target to improve muscle function in LAMA2-CMD. Here, we have investigated whether the multifunctional medication metformin could be used to reduce disease in the dy 2 J /dy 2J mouse model of LAMA2-CMD. First, we show gender disparity for several pathological hallmarks of LAMA2-CMD. Second, we demonstrate that metformin treatment significantly increases weight gain and energy efficiency, enhances muscle function and improves skeletal muscle histology in female dy 2J /dy 2J mice (and to a lesser extent in dy 2J /dy 2J males). Thus, our current data suggest that metformin may be a potential future supportive treatment that improves many of the pathological characteristics of LAMA2-CMD." @default.
• W2899154927 created "2018-11-09" @default.
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• W2899154927 date "2018-11-02" @default.
• W2899154927 modified "2023-10-16" @default.
• W2899154927 title "Effects of metformin on congenital muscular dystrophy type 1A disease progression in mice: a gender impact study" @default.
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• W2899154927 doi "https://doi.org/10.1038/s41598-018-34362-2" @default.
• W2899154927 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6214987" @default.
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• W2899154927 hasPublicationYear "2018" @default.
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