Mitochondrial abnormalities in progressive external ophthalmoplegia.
Three cases of chronic progressive external ophthalmoplegia demonstrated ragged-red fibers. Histochemical stains showed that the ragged-red fiber appearance was produced by abnormal mitochondria in the muscle. Electron microscopic studies demonstrated the ultrastructural origin of the intramitochondrial inclusions seen in ragged-red fibers. The associated abnormalities in lactic acid metabolism possibly represent an underlying generalized defect in metabolism. This evidence suggests that chronic progressive external ophthalmoplegia is a diffuse disease of the mitochondria.
D000328:Adult; D002239:Carbohydrate Metabolism, Inborn Errors / Q000150:complications* / Q000473:pathology; D002908:Chronic Disease; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D007773:Lactates / Q000097:blood / Q000378:metabolism*; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000473:pathology* / Q000648:ultrastructure; D009132:Muscles / Q000473:pathology; D009886:Ophthalmoplegia / Q000209:etiology* / Q000473:pathology
D007773:Lactates