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

Handschin, C. and Kobayashi, Y. M. and Chin, S. and Seale, P. and Campbell, K. P. and Spiegelman, B. M.. (2007) PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Genes & development, Vol. 21, H. 7. pp. 770-783.

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Official URL: http://edoc.unibas.ch/dok/A5258712

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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.
Faculties and Departments:05 Faculty of Science > Departement Biozentrum > Growth & Development > Growth & Development (Handschin)
03 Faculty of Medicine > Departement Biomedizin > Associated Research Groups > Pharmakologie (Handschin)
UniBasel Contributors:Handschin, Christoph
Item Type:Article, refereed
Article Subtype:Research Article
Bibsysno:Link to catalogue
Publisher:Cold Spring Harbor Laboratory
ISSN:0890-9369
Note:Publication type according to Uni Basel Research Database: Journal article
Language:English
Identification Number:
Last Modified:31 Dec 2015 10:42
Deposited On:22 Mar 2012 13:20

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