Disruption of skeletal muscle mitochondrial network genes and miRNAs in amyotrophic lateral sclerosis

  title={Disruption of skeletal muscle mitochondrial network genes and miRNAs in amyotrophic lateral sclerosis},
  author={Aaron P Russell and Shogo Wada and Lodovica Vergani and M. Benjamin Hock and Takayuki Akimoto},
  journal={Neurobiology of Disease},
Skeletal muscle mitochondrial dysfunction is believed to play a role in the progression and severity of amyotrophic lateral sclerosis (ALS). The regulation of transcriptional co-activators involved in mitochondrial biogenesis and function in ALS is not well known. When compared with healthy control subjects, patients with ALS, but not neurogenic disease (ND), had lower levels of skeletal muscle peroxisome proliferator-activated receptor γ coactivator-1α (PGC-1α) mRNA and protein and estrogen… CONTINUE READING


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Publications referenced by this paper.
Showing 1-10 of 75 references

Elevated PGC1 alpha activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS

S. Da Cruz
Cell Metab . • 2012

Reduced exercise capacity in early-stage amyotrophic lateral sclerosis: Role of skeletal muscle.

Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases • 2012

Hind limb muscle atrophy precedes cerebral neuronal degeneration in G 93 ASOD 1 mouse model of amyotrophic lateral sclerosis : a longitudinal MRI study

M. Mensink
Exp . Neurol . • 2011

Striated muscle activator of Rho signalling (STARS) is a PGC1α/ERRα target gene and is upregulated in human skeletal muscle following endurance exercise

Wallace, M.A
J. Physiol • 2011

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