Skeletal muscle oxidative capacity in amyotrophic lateral sclerosis

Terence E. Ryan, Melissa L. Erickson, Ajay Verma, Juan Chavez, Michael H. Rivner, Kevin K. Mccully

Research output: Contribution to journalArticlepeer-review

24 Scopus citations


Introduction: Mitochondrial dysfunction in the motor neuron has been suspected in amyotrophic lateral sclerosis (ALS). If mitochondrial abnormalities are also found in skeletal muscle, assessing skeletal muscle could serve as an important biomarker of disease progression. Methods: Using 31P magnetic resonance (31P-MRS) and near infrared (NIRS) spectroscopy, we compared the absolute values and reproducibility of skeletal muscle oxidative capacity in people with ALS (n=6) and healthy adults (young, n=7 and age-matched, n=4). Results: ALS patients had slower time constants for phosphocreatine (PCr) and muscle oxygen consumption (mVO2) compared with young, but not age-matched controls. The coefficient of variation for the time constant was 10% (SD=2.8%) and 17% (SD=6.2%) for PCr and mVO2, respectively. Conclusions: People with ALS had, on average, a small but not statistically significant, impairment in skeletal muscle mitochondrial function measured by both 31P-MRS and NIRS. Both methods demonstrated good reproducibility.

Original languageEnglish (US)
Pages (from-to)767-774
Number of pages8
JournalMuscle and Nerve
Issue number5
StatePublished - Nov 1 2014


  • MRS
  • Mitochondrial bioenergetics
  • Mitochondrial function
  • Motor neuron disease
  • NIRS

ASJC Scopus subject areas

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


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