Article
Glutathione depletion in antioxidant defense of differentiated NT2-LHON cybrids.
Neurobiology of disease - 1 Mar 2007
Schoeler S, Winkler-Stuck K, Szibor R, Haroon M F, Gellerich F N, Chamaon K, Mawrin C, Kirches E
Abstract excerpt
The mechanism of retinal ganglion cell loss in Leber's hereditary optic neuropathy (LHON) is still uncertain, and a role of enhanced superoxide production by the mutant mitochondrial complex I has been hypothesized. In the present study, it was shown that LHON cybrids, carrying the np11778 mutation, became selectively more H(2)O(2) sensitive compared with the parental cell line only following short-term retinoic...
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