Effects of metformin on congenital muscular dystrophy type 1A disease progression in mice: a gender impact study.

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^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 weigh

SUBMITTER: Fontes-Oliveira CC 

PROVIDER: S-EPMC6214987 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature