INCREASED DENSITY OF MITOCHONDRIA ISOLATED FROM THE SKELETAL MUSCLE OF A PATIENT WITH MITOCHONDRIAL MYOPATHY

Mitochondria from the muscle of a patient with mitochondrial myopathy contained paracrystalline inclusion bodies. To characterize the mitochondrial fraction containing the crystalline materials, the muscle homogenate was subjected to a Ficoll density gradient centrifugation. The mitochondrial fraction was identified by immunoblotting with specific antisera against the F1-ATPase β-subunit and lipoamide acetyltransferase of the pyruvate dehydrogenase complex. The mitochondria from the patient had a density higher than in mitochondria from controls. This method is useful for isolating abnormal mitochondria from muscle specimens of about 0.1 ｇ.