Biochemical studies of patients with Cuban epidemic neuropathy.
In 1992-1994, a disorder known as the epidemic neuropathy afflicted more than 50,000 Cubans. Three different forms of the illness were identified: epidemic optic neuropathy, peripheral neuropathy and mixed optic and peripheral neuropathy. The causes are still unknown. Skeletal muscle biopsy samples were analyzed by standard histological techniques and by biochemical assays. Elevated activities of citrate synthase, a non-respiratory-chain mitochondrial matrix enzyme, suggested possible mitochondrial proliferation in 7 of the 8 patients. Nicotinamide adenine dinucleotide phosphate (NADP(+)) levels were higher in the patients than in the controls (p = 0.04). Levels of nicotinamide adenine dinucleotide (NAD) and the reduced compounds NADH and NADPH were comparable in patients and controls. Elevations of succinate dehydrogenase and citrate synthase activities and high NADP(+) levels suggest that alterations of mitochondrial functions may be associated with this disorder.
Rodríguez-Hernandez
M
M
Department of Neurogenetics, Institute of Neurology and Neurosurgery, Havana, Cuba.
Hirano
M
M
Naini
A
A
Santiestéban
R
R
D000328:Adult; D002950:Citrate (si)-Synthase / Q000378:metabolism; D003462:Cuba / Q000453:epidemiology; D003576:Electron Transport Complex IV / Q000378:metabolism; D006801:Humans; D008875:Middle Aged; D028361:Mitochondrial Diseases / Q000201:enzymology* / Q000453:epidemiology; D018482:Muscle, Skeletal / Q000201:enzymology; D009249:NADP / Q000378:metabolism; D009901:Optic Nerve Diseases / Q000201:enzymology* / Q000453:epidemiology; D010088:Oxidoreductases / Q000378:metabolism*; D010523:Peripheral Nervous System Diseases / Q000201:enzymology* / Q000453:epidemiology; D013384:Succinate Cytochrome c Oxidoreductase / Q000378:metabolism; D013385:Succinate Dehydrogenase / Q000378:metabolism
D009249:NADP; D010088:Oxidoreductases; D013384:Succinate Cytochrome c Oxidoreductase; D013385:Succinate Dehydrogenase; D003576:Electron Transport Complex IV; D002950:Citrate (si)-Synthase