Glutathione depletion in antioxidant defense of differentiated NT2-LHON cybrids.

  title={Glutathione depletion in antioxidant defense of differentiated NT2-LHON cybrids.},
  author={Susanne Schoeler and Kirstin Winkler-Stuck and Reinhard Szibor and Mohammad Fahad Haroon and Frank Norbert Gellerich and Kathrin Chamaon and Christian Mawrin and E. J. F. Kirches},
  journal={Neurobiology of disease},
  volume={25 3},
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 acid differentiation. They contained a decreased cellular glutathione pool (49%, p… CONTINUE READING