Antioxidants Reduce Muscular Dystrophy in the dy^2J/dy^2J Mouse Model of Laminin α2 Chain-Deficient Muscular Dystrophy.

ABSTRACT: Congenital muscular dystrophy with laminin α2 chain-deficiency (LAMA2-CMD) is a severe neuromuscular disorder without a cure. Using transcriptome and proteome profiling as well as functional assays, we previously demonstrated significant metabolic impairment in skeletal muscle from LAMA2-CMD patients and mouse models. Reactive oxygen species (ROS) increase when oxygen homeostasis is not maintained and, here, we investigate whether oxidative stress indeed is involved in the pathogenesis of LAMA2-CMD. We also analyze the effects of two antioxidant molecules, N-acetyl-L-cysteine (NAC) and vitamin E, on disease progression in the dy^2J/dy^2J mouse model of LAMA2-CMD. We demonstrate increased ROS levels in LAMA2-CMD mouse and patient skeletal muscle. Furthermor

SUBMITTER: Harandi VM 

PROVIDER: S-EPMC7139799 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature