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

Huiyun Liang, Walter F. Ward, Youngmok C. Jang, Arunabh Bhattacharya, Alex F. Bokov, Yan Li, Amanda Jernigan, Arlan Richardson, Holly van Remmen

Research output: Contribution to journalArticlepeer-review

54 Scopus citations


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 6 3 days vs. 274 6 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.

Original languageEnglish (US)
Pages (from-to)947-956
Number of pages10
JournalMuscle and Nerve
Issue number6
StatePublished - Dec 2011


  • Amyotrophic lateral sclerosis
  • Excitatory amino acid transporter protein 2 (EAAT2)
  • Motor neuron
  • Neuromuscular junction
  • PGC-1α transgenic mice

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)


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