Defective pattern of mitochondrial respiratory enzymes in mitochondrial myopathy

Mitochondrial myopathies consist of miscellaneous entities characterized by muscular atrophy and morphologically abnormal mitochondria. Defects in the oxidative phosphorylation system have been recognized as main causes of mitochondrial myopathy. This paper reports immunochemical and immunohistochemical studies which were performed on the skeletal muscle and fibroblasts of these patients in order to understand the molecular defect of this particular disorder