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Muscle Nerve. 2011 Dec;44(6):947-56. doi: 10.1002/mus.22217.

PGC-1α protects neurons and alters disease progression in an amyotrophic lateral sclerosis mouse model.

Author information

1
Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, Texas, USA.

Abstract

INTRODUCTION:

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease. We sought to determine whether peroxisome proliferator-activated receptor γ coactivator 1α (PGC-1α) would have a beneficial effect on this disease.

METHODS:

PGC-1α transgenic mice were crossed with SOD1 mutant G93A DL mice.

RESULTS:

We observed a moderate but non-significant increase in average lifespan in PGC-1α/G93A DL mice, as compared with G93A DL mice (292 ± 3 days vs. 274 ± 7 days). Although the onset of ALS was not altered, progression of the disease was significantly slower (≈34% increase in duration) in the PGC-1α/G93A DL mice. These mice also exhibited markedly improved performance on the rotarod test, and the improved motor activity was associated with a decreased loss of motor neurons and less degeneration of neuromuscular junctions.

CONCLUSION:

A sustained level of excitatory amino acid transporter protein 2 (EAAT2) in astrocytes of the PGC-1α/G93A DL mice may contribute to neuronal protection.

PMID:
22102466
DOI:
10.1002/mus.22217
[Indexed for MEDLINE]

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