Fatty acid metabolism and mitochondrial proteins in the C57BL/6J-dy2Jdy2J dystrophic mice.
Measurements of [1-14C]oleoyl CoA oxidation in homogenates and isolated mitochondria of skeletal muscles, hearts and livers from dystrophic C57BL/6J mice and unaffected litter-mates indicate that rates are lower in dystrophic mitochondria, but are comparable in whole homogenates. These findings are not due to differences in the endogenous concentrations of the fatty CoA esters. Qualitative and quantitative differences in the molecular weight distribution of the proteins in the mitochondria and post-mitochondrial supernatants are found in the skeletal muscles with increases in the post-mitochondrial supernatant and decreases in the mitochondria in dystrophic samples. We suggest that these results are due to increased leakiness of the mitochondrial membranes in dystrophic muscles. Such differences were not observed in preparations from normal animals.
D000818:Animals; D005227:Fatty Acids / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D008929:Mitochondria, Heart / Q000378:metabolism*; D008930:Mitochondria, Liver / Q000378:metabolism*; D008931:Mitochondria, Muscle / Q000378:metabolism*; D008970:Molecular Weight; D009137:Muscular Dystrophy, Animal / Q000378:metabolism*; D009928:Organ Specificity; D011506:Proteins / Q000302:isolation & purification / Q000378:metabolism*; D012016:Reference Values
D005227:Fatty Acids; D011506:Proteins