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: Yu Kitaoka, Daniel I Ogborn, Nicholas J Mocellin, Uwe Schlattner, Mark A Tarnopolsky
• Journal: Molecular genetics and metabolism (Mol Genet Metab) Vol. 108 Issue 4 Pg. 259-62 (Apr 2013) ISSN: 1096-7206 [Electronic] United States
• PMID: 23434346 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2013 Elsevier Inc. All rights reserved.

Chemical References

• Membrane Transport Proteins
• Monocarboxylic Acid Transporters
• Creatine Kinase, Mitochondrial Form

Topics

• Creatine Kinase, Mitochondrial Form (metabolism)
• Female
• Glycogen Storage Disease Type V (metabolism)
• Glycogenolysis
• Humans
• Male
• Membrane Transport Proteins (metabolism)
• Middle Aged
• Mitochondria (enzymology, metabolism)
• Monocarboxylic Acid Transporters (metabolism)
• Muscle, Skeletal (metabolism)
• Muscular Diseases (metabolism)