title: Studies on mitochondria from dystrophic skeletal muscle of mice.
abstract: Mitochondrial respiration and oxidative phosphorylation were compared in normal and dystrophic mouse skeletal muscles. To obtain the maximum respiration control ratio (RCR) and adenosine diphosphate/oxygen (ADP/O) ratio from isolated muscle mitochondria, it is found that there is an advantage in having a low concentration of proteinase and EGTA present in the medium during preparation of mitochondria by centrifugation fractionation. Using pyruvate, acetylcarnitine, and palmitylcarnitine as substrates for oxidation, a highly significant reduction (40-60%) is shown in oxygen uptake by dystrophic muscle mitochondria as compared to normal muscle mitochondria. Studies of the integrity of the oxidative phosphorylation apparatus in these samples showed that there is a reduction of the RCR and ADP/O ratio in dystrophic muscle mitochondria as compared to normal muscle mitochondria.
mesh_terms: D000244:Adenosine Diphosphate / Q000378:metabolism; D000818:Animals; D002332:Carnitine O-Acetyltransferase / Q000378:metabolism; D004533:Egtazic Acid / Q000494:pharmacology; D005969:Glutamate Dehydrogenase / Q000378:metabolism; D066298:In Vitro Techniques; D051379:Mice; D008931:Mitochondria, Muscle / Q000378:metabolism*; D009137:Muscular Dystrophy, Animal / Q000378:metabolism*; D010101:Oxygen Consumption; D013379:Substrate Specificity; D013385:Succinate Dehydrogenase / Q000378:metabolism
chemical_list: D004533:Egtazic Acid; D000244:Adenosine Diphosphate; D013385:Succinate Dehydrogenase; D005969:Glutamate Dehydrogenase; D002332:Carnitine O-Acetyltransferase