PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy

Author:

Handschin Christoph,Kobayashi Yvonne M.,Chin Sherry,Seale Patrick,Campbell Kevin P.,Spiegelman Bruce M.

Abstract

The coactivator PGC-1α mediates key responses of skeletal muscle to motor nerve activity. We show here that neuregulin-stimulated phosphorylation of PGC-1α and GA-binding protein (GABP) allows recruitment of PGC-1α to the GABP complex and enhances transcription of a broad neuromuscular junction gene program. Since a subset of genes controlled by PGC-1α and GABP is dysregulated in Duchenne muscular dystrophy (DMD), we examined the effects of transgenic PGC-1α in muscle of mdx mice. These animals show improvement in parameters characteristic of DMD, including muscle histology, running performance, and plasma creatine kinase levels. Thus, control of PGC-1α levels in skeletal muscle could represent a novel avenue to prevent or treat DMD.

Publisher

Cold Spring Harbor Laboratory

Subject

Developmental Biology,Genetics

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