Journal article
PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy
Genes & Development, Vol.21, p.770
2007
DOI: 10.1101/gad.1525107
PMCID: PMC1838529
PMID: 17403779
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.
Details
- Title: Subtitle
- PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy
- Creators
- Christoph HandschinYvonne M KobayashiSherry ChinPatrick SealeKevin P CampbellBruce M Spiegelman
- Resource Type
- Journal article
- Publication Details
- Genes & Development, Vol.21, p.770
- Publisher
- Cold Spring Harbor Laboratory Press
- DOI
- 10.1101/gad.1525107
- PMID
- 17403779
- PMCID
- PMC1838529
- ISSN
- 0890-9369
- eISSN
- 1549-5477
- Alternative title
- PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy
- Language
- English
- Date published
- 2007
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984020706202771
Metrics
20 Record Views