Glutathione depletion in antioxidant defense of differentiated NT2-LHON cybrids
| Autor: | Schoeler S, Kathrin Chamaon, Elmar Kirches, M.F. Haroon, R. Szibor, F.N. Gellerich, C. Mawrin, Kirstin Winkler-Stuck |
|---|---|
| Rok vydání: | 2006 |
| Předmět: |
GPX1
GPX3 Genotype Glutathione reductase Cybrid Antineoplastic Agents Tretinoin Optic Atrophy Hereditary Leber GPX6 Antioxidants lcsh:RC321-571 Superoxide dismutase chemistry.chemical_compound LHON Glutaredoxin Cell Line Tumor Antioxidant defense Humans Point Mutation lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry chemistry.chemical_classification Glutathione Peroxidase Electron Transport Complex I biology Superoxide Dismutase Glutathione peroxidase Teratoma ROS Cell Differentiation Glutathione Hydrogen Peroxide Oxidants Molecular biology Mitochondria Glutathione Reductase Neurology chemistry Biochemistry biology.protein |
| Zdroj: | Neurobiology of Disease, Vol 25, Iss 3, Pp 536-544 (2007) |
| ISSN: | 0969-9961 |
| Popis: | 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 ≤ 0.05), despite 1.5-fold enhanced expression of the regulatory subunit of γ-glutamylcysteine synthetase ( p ≤ 0.05). This points to a reduction of the capacity to detoxify H 2 O 2 and to changes in thiol redox potential. The activity of the H 2 O 2 degrading enzyme glutathione peroxidase (GPx) and the activities of glutathione reductase (GR) and superoxide dismutase (SOD) were unaffected. |
| Databáze: | OpenAIRE |
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