Journal
GENES & DEVELOPMENT
Volume 21, Issue 7, Pages 770-783Publisher
COLD SPRING HARBOR LAB PRESS, PUBLICATIONS DEPT
DOI: 10.1101/gad.1525107
Keywords
PGC-1; neuromuscular junction; GA-binding protein; Duchenne muscular dystrophy; transcriptional regulation
Categories
Funding
- NIDDK NIH HHS [DK61562, DK54477, R56 DK054477, R01 DK054477, R01 DK061562] Funding Source: Medline
Ask authors/readers for more resources
The coactivator PGC-1 alpha mediates key responses of skeletal muscle to motor nerve activity. We show here that neuregulin-stimulated phosphorylation of PGC-1 alpha and GA-binding protein (GABP) allows recruitment of PGC-1 alpha to the GABP complex and enhances transcription of a broad neuromuscular junction gene program. Since a subset of genes controlled by PGC-1 alpha and GABP is dysregulated in Duchenne muscular dystrophy (DMD), we examined the effects of transgenic PGC-1 alpha 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-1 alpha levels in skeletal muscle could represent a novel avenue to prevent or treat DMD.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available