title: Monocarboxylate transporters and mitochondrial creatine kinase protein content in McArdle disease.
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: Department of Pediatrics, McMaster University, 1200 Main Street West, Hamilton, Ontario, Canada L8N 3Z5. kitaoka@mcmaster.ca
authors: Yu
authors: Y
authors: Kitaoka
authors: Daniel I
authors: DI
authors: Ogborn
authors: Nicholas J
authors: NJ
authors: Mocellin
authors: Uwe
authors: U
authors: Schlattner
authors: Mark A
authors: MA
authors: Tarnopolsky
mesh_terms: D052281:Creatine Kinase, Mitochondrial Form / Q000378:metabolism*; D005260:Female; D006012:Glycogen Storage Disease Type V / Q000378:metabolism*; D050261:Glycogenolysis; D006801:Humans; D008297:Male; D026901:Membrane Transport Proteins / Q000378:metabolism; D008875:Middle Aged; D008928:Mitochondria / Q000201:enzymology / Q000378:metabolism; D027501:Monocarboxylic Acid Transporters / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000378:metabolism; D009135:Muscular Diseases / Q000378:metabolism
chemical_list: D026901:Membrane Transport Proteins; D027501:Monocarboxylic Acid Transporters; D052281:Creatine Kinase, Mitochondrial Form