Journal
SCIENTIFIC REPORTS
Volume 8, Issue -, Pages -Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/s41598-018-34362-2
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Funding
- Anna and Edwin Berger Foundation
- Anna-Greta Crafoord Foundation for Rheumatology Research
- Conselho Nacional de Desenvolvimento Cientifico e Tecnologico (CNPq)
- Crafoord Foundation
- Fanny Ekdahl Foundation
- Greta and Johan Kock Foundation
- Jane and Dan Olsson Foundation
- Lisa and Johan Gronberg Foundation
- Neuroforbundet
- Olle Engkvist Byggmastare Foundation
- Royal Physiographic Society in Lund
- Swedish Research Council
- Tore Nilsson Foundation
- Osterlund Foundation
- Association Francaise contre les Myopathies
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Congenital muscular dystrophy with laminin alpha 2 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(2J)/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.
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