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Article

Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber's hereditary optic neuropathy.

The FEBS journal - 1 Mar 2005

Floreani Maura, Napoli Eleonora, Martinuzzi Andrea, Pantano Giorgia, De Riva Valentina, Trevisan Roberta, Bisetto Elena, Valente Lucia, Carelli Valerio, Dabbeni-Sala Federica

Abstract excerpt

Oxidative stress and imbalance between free radical generation and detoxification may play a pivotal role in the pathogenesis of Leber's hereditary optic neuropathy (LHON). Mitochondria, carrying the homoplasmic 11778/ND4, 3460/ND1 and 14484/ND6 mtDNA point mutations associated with LHON, were used to generate osteosarcoma-derived cybrids. Enhanced mitochondrial production of reactive oxygen species has recently...

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Identifiers

PMID: 15720387
DOI: 10.1111/j.1742-4658.2004.04542.x

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber's hereditary optic neuropathy.PMID 15720387Read ↗

Oxidative Stress Profile in Genetically Confirmed Cases of Leber's Hereditary Optic Neuropathy.Semantic proximity Read ↗

Leber's hereditary optic neuropathy (LHON) pathogenic mutations induce mitochondrial-dependent apoptotic death in transmitochondrial cells incubated with galactose medium.Semantic proximity Read ↗

alpha-Tocopherol/lipid ratio in blood is decreased in patients with Leber's hereditary optic neuropathy and asymptomatic carriers of the 11778 mtDNA mutation.Semantic proximity Read ↗

Glutathione depletion in antioxidant defense of differentiated NT2-LHON cybrids.Semantic proximity Read ↗

Leber's hereditary optic neuropathy: biochemical effect of 11778/ND4 and 3460/ND1 mutations and correlation with the mitochondrial genotype.Semantic proximity Read ↗

[Evaluation of serum levels of SOD and MDA in patients with Leber's hereditary optic neuropathy carrying the mitochondrial DNA G11778A mutation].Semantic proximity Read ↗

Oestrogens ameliorate mitochondrial dysfunction in Leber’s hereditary optic neuropathySemantic proximity Read ↗

Evidence for Detrimental Cross Interactions between Reactive Oxygen and Nitrogen Species in Leber's Hereditary Optic Neuropathy Cells.Semantic proximity Read ↗

Galactose-replacement unmasks the biochemical consequences of the G11778A mitochondrial DNA mutation of LHON in patient-derived fibroblasts.Semantic proximity Read ↗

Biochemical evidence for a mitochondrial genetic modifier in the phenotypic manifestation of Leber's hereditary optic neuropathy-associated mitochondrial DNA mutation.Semantic proximity Read ↗

Combination treatment with antioxidants and creatine alleviates common and variant-specific mitochondrial impairments in Leber's hereditary optic neuropathy patient-derived fibroblasts.Semantic proximity Read ↗

Assocation Between Leber's Hereditary Optic Neuropathy and MT-ND1 3460G>A Mutation-Induced Alterations in Mitochondrial Function, Apoptosis, and Mitophagy.Semantic proximity Read ↗

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