PPARδ modulation rescues mitochondrial fatty acid oxidation defects in the mdx model of muscular dystrophy.

ABSTRACT

Duchenne muscular dystrophy (DMD) is a recessive, fatal X-linked disease that is characterized by progressive skeletal muscle wasting due to the absence of dystrophin, which is an a essential protein that bridges the inner cytoskeleton and extra-cellular matrix. This study set out to characterize the mitochondria in primary muscle satellite cell derived myoblasts from mdx mice and wild type control mice. Compared to wild type derived cells the mdx derived cells have reduced mitochondrial bioenergetics and have fewer mitochondria. Here, we demonstrate that a novel PPARδ modulator improves mitochondrial function in the mdx mice, which supports that modulating PPARδ may be therapeutically beneficial in DMD patients.