Defective Oxidative Phosphorylation in Hereditary Myocardiopathy in the Syrian Hamster

Abstract
Biochemical studies have been performed on the hearts of hamsters afflicted with a hereditary, fatal myocardiopathy and muscular dystrophy. In sarcosomes from myopathic hearts phosphorus uptake and phosphorus/oxygen ratios were depressed, while oxygen uptake was normal. The enhanced rate of glycolysis in slices from myopathic hearts could be explained by changes in high energy phosphate compound metabolism. The relationship between the biochemical changes and the mechanical failure of the heart is discussed.