PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy
Top Cited Papers
- 1 April 2007
- journal article
- Published by Cold Spring Harbor Laboratory in Genes & Development
- Vol. 21 (7) , 770-783
- https://doi.org/10.1101/gad.1525107
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.Keywords
This publication has 55 references indexed in Scilit:
- Advances in Male ContraceptionEndocrine Reviews, 2008
- PGC-1α protects skeletal muscle from atrophy by suppressing FoxO3 action and atrophy-specific gene transcriptionProceedings of the National Academy of Sciences, 2006
- Molecular architecture of the neuromuscular junctionMuscle & Nerve, 2005
- Nutritional Regulation of Hepatic Heme Biosynthesis and Porphyria through PGC-1αCell, 2005
- Human muscle gene expression responses to endurance training provide a novel perspective on Duchenne muscular dystrophyThe FASEB Journal, 2005
- PGC-1α Deficiency Causes Multi-System Energy Metabolic Derangements: Muscle Dysfunction, Abnormal Weight Control and Hepatic SteatosisPLoS Biology, 2005
- Involvement of PPARγ co-activator-1, nuclear respiratory factors 1 and 2, and PPARα in the adaptive response to endurance exerciseProceedings of the Nutrition Society, 2004
- Phosphorylation-Elicited Quaternary Changes of GA Binding Protein in Transcriptional ActivationMolecular and Cellular Biology, 2003
- Transcriptional co-activator PGC-1α drives the formation of slow-twitch muscle fibresNature, 2002
- Structure and function of the neuromuscular junction in young adultmdx miceJournal of Neurocytology, 1991