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      Biochemical Studies of Patients with Cuban Epidemic Neuropathy

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          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<sup>+</sup>) 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<sup>+</sup> levels suggest that alterations of mitochondrial functions may be associated with this disorder.

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          High-performance liquid chromatography analysis of oxidized and reduced pyridine dinucleotides in specific brain regions.

          An ultrasensitive HPLC method has been developed for measuring NADP+, NADPH, NAD+, and NADH. A simple, rapid reaction of the oxidized nucleotides with cyanide in basic solution leads to two stable fluorescent products and allows all four nucleotides to be separated and quantitated on one chromatogram. Furthermore, only one extraction is needed, rather than prior procedures which require one acid extraction (for oxidized species) and one basic extraction (for reduced species). This method is particularly useful in quantitating pyridine dinucleotides in rodent brain, where no current method is adequate to quantitate the small amounts contained in various brain regions. The assay is sensitive enough to measure individual brain regions down to 10 mg of tissue. Due to the involvement of NAD(P)H enzymatic systems in combating oxidative stress it is important to be able to assess levels regionally in brain diseases.
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            Functional alterations of the mitochondrially encoded ND4 subunit associated with Leber's hereditary optic neuropathy.

            Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease associated with point mutations in mitochondrial DNA. The most frequent of these mutations is the G-to-A substitution at nucleotide position 11,778 which changes an evolutionarily conserved arginine with a histidine at position 340 in subunit ND4 of NADH:ubiquinone reductase (respiratory complex I). We report that this amino acid substitution alters the affinity of complex I for the ubiquinone substrate and induces resistance towards its potent inhibitor rotenone in mitochondria of LHON patients. Such changes could reflect a substantial loss in the energy conserving function of NADH:ubiquinone reductase and thus explain the pathological effect of the ND4/11,778 mutation.

              Author and article information

              Ophthalmic Res
              Ophthalmic Research
              S. Karger AG
              December 2001
              22 November 2001
              : 33
              : 6
              : 310-313
              Departments of aNeurogenetics and bNeuro-Ophthalmology, Institute of Neurology and Neurosurgery, Havana, Cuba; cDepartment of Neurology, College of Physicians and Surgeons, Columbia University, New York, N.Y., USA
              55686 Ophthalmic Res 2001;33:310–313
              © 2001 S. Karger AG, Basel

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              Page count
              Tables: 2, References: 17, Pages: 4
              Original Paper


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