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

PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Research Abstract Details 

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PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Abstract Text:

Christoph Handschin,Yvonne M Kobayashi,Sherry Chin,Patrick Seale,Kevin P Campbell,Bruce M Spiegelman,

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.

PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Publishing Authors By Initials

C Handschin,YM Kobayashi,S Chin,P Seale,KP Campbell,BM Spiegelman,

For similar investigative techniques: genetic techniques: gene transfer techniques: transfection research abstracts see: investigative techniques: genetic techniques: gene transfer techniques: transfection research

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PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Genes & development

VOLUME: 21

Page Numbers: 770-83

Journal Abbreviation: Genes Dev.

ISSN: 0890-9369

DAY: 1

MONTH: Apr

YEAR: 2007

PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 8711660

PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Keywords Mesh Terms:

KEYWORDS: Transfection

MESH TERMS: metabolism

Chemical & Substance for Abstract: PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Information

Substance Name: Trans-Activators

Registry Number: 0

Grant and Affiliation Information for PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy.

AFFILIATION: Dana-Farber Cancer Institute and Department of Cell Biology, Harvard Medical School, Boston, Massachusetts 02115, USA.

Country: United States

AGENCY: United States NIDDK

GRANT: DK61562

ACRONYM: DK

MEDLINETA: Genes Dev

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