Monocarboxylate transporters and mitochondrial creatine kinase protein content in McArdle disease Academic Article uri icon

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abstract

  • McArdle disease (MD) is a metabolic myopathy due to myophosphorylase deficiency. We examined monocarboxylate transporters (MCT) and creatine kinase (CK) protein content in skeletal muscle from MD patients and age-matched controls to evaluate potential cellular adaptations that compensate for the loss of glycogenolysis. Our findings of higher MCT1 and mitochondrial CK suggest that proteins related to extra-muscular fuel uptake and intra-muscular energy transduction are up-regulated without change in mitochondrial mass in MD patients.

authors

  • Kitaoka, Yu
  • Ogborn, Daniel I
  • Mocellin, Nicholas J
  • Schlattner, Uwe
  • Tarnopolsky, Mark

publication date

  • April 2013