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

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.
Source: Mitochondrion - Category: Biochemistry Source Type: research