Adenylosuccinic acid therapy ameliorates murine Duchenne Muscular Dystrophy.

ABSTRACT: Arising from the ablation of the cytoskeletal protein dystrophin, Duchenne Muscular Dystrophy (DMD) is a debilitating and fatal skeletal muscle wasting disease underpinned by metabolic insufficiency. The inability to facilitate adequate energy production may impede calcium (Ca²⁺) buffering within, and the regenerative capacity of, dystrophic muscle. Therefore, increasing the metabogenic potential could represent an effective treatment avenue. The aim of our study was to determine the efficacy of adenylosuccinic acid (ASA), a purine nucleotide cycle metabolite, to stimulate metabolism and buffer skeletal muscle damage in the mdx mouse model of DMD. Dystrophin-positive control (C57BL/10) and dystrophin-deficient mdx mice were treated with ASA (3000 µg.mL^-1) in drinking

SUBMITTER: Timpani CA 

PROVIDER: S-EPMC6981178 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature