Sarcolipin haploinsufficiency prevents dystrophic cardiomyopathy in mdx mice.

ABSTRACT: Sarcolipin (SLN) is an inhibitor of sarco/endoplasmic reticulum (SR) Ca²⁺-ATPase (SERCA) and expressed at high levels in the ventricles of animal models for and patients with Duchenne muscular dystrophy (DMD). The goal of this study was to determine whether the germline ablation of SLN expression improves cardiac SERCA function and intracellular Ca²⁺ (Ca²⁺_i) handling and prevents cardiomyopathy in the mdx mouse model of DMD. Wild-type, mdx, SLN-haploinsufficient mdx (mdx:sln^+/-), and SLN-deficient mdx (mdx:sln^-/-) mice were used for this study. SERCA function and Ca²⁺_i handling were determined by Ca²⁺ uptake assays and by measuring single-cell Ca

SUBMITTER: Mareedu S 

PROVIDER: S-EPMC7847070 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature