| First Author | Handschin C | Year | 2007 |
| Journal | Genes Dev | Volume | 21 |
| Issue | 7 | Pages | 770-83 |
| PubMed ID | 17403779 | Mgi Jnum | J:120376 |
| Mgi Id | MGI:3706458 | Doi | 10.1101/gad.1525107 |
| Citation | Handschin C, et al. (2007) PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Genes Dev 21(7):770-83 |
| abstractText | 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. |
