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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-2299

Handschin, C; Kobayashi, Y M; Chin, S; Seale, P; Campbell, K P; Spiegelman, B M (2007). PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Genes and Development, 21(7):770-783.

Accepted Version


The coactivator PGC-1alpha mediates key responses of skeletal muscle to motor nerve activity. We show here that neuregulin-stimulated phosphorylation of PGC-1alpha and GA-binding protein (GABP) allows recruitment of PGC-1alpha to the GABP complex and enhances transcription of a broad neuromuscular junction gene program. Since a subset of genes controlled by PGC-1alpha and GABP is dysregulated in Duchenne muscular dystrophy (DMD), we examined the effects of transgenic PGC-1alpha 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-1alpha levels in skeletal muscle could represent a novel avenue to prevent or treat DMD.

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Institute of Physiology
07 Faculty of Science > Institute of Physiology
DDC:570 Life sciences; biology
Deposited On:18 Mar 2008 10:04
Last Modified:27 Nov 2013 18:24
Publisher:Cold Spring Harbor Laboratory Press
Publisher DOI:10.1101/gad.1525107
PubMed ID:17403779
Citations:Web of Science®. Times Cited: 120
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Scopus®. Citation Count: 122

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