α-tocopherol/lipid ratio in blood is decreased in patients with Leber's hereditary optic neuropathy and asymptomatic carriers of the 11778 mtDNA mutation

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Abstract

Objectives - Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease characterised by acute or subacute bilateral visual loss in young patients. The primary aetiological event is a mutation in the mitochondrial genome (mtDNA) affecting in most cases mtDNA-encoded subunits of the respiratory chain NADH: coenzyme Q oxidoreductase (complex I). The impaired function of complex I leads to a decline in mitochondrial energy production and enhances free radical generation. Methods - The concentrations of some non-enzymatic antioxidants (α-tocopherol, β-carotene, lycopene, glutathione, free sulphydryl groups) and the lipid peroxides in the blood of patients with LHON, carriers with homoplasmic DNA mutation at 11 778, and controls were investigated using high performance liquid chromatography and spectrophotometric methods to assess the function of their antioxidant defence systems. Results - The α-tocopherol/cholesterol+ triglyceride ratio was significantly reduced (p

Original languageEnglish
Pages (from-to)359-362
Number of pages4
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume70
Issue number3
DOIs
Publication statusPublished - Mar 2001

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Leber's Hereditary Optic Atrophy
Tocopherols
Mitochondrial DNA
Antioxidants
Lipids
Mutation
Ubiquinone
Mitochondrial Genome
Lipid Peroxides
Acute Disease
Carotenoids
Electron Transport
NAD
Free Radicals
Glutathione
Oxidoreductases
Triglycerides
Cholesterol
High Pressure Liquid Chromatography
DNA

Keywords

  • α-tocopherol
  • Free radicals
  • Leber's hereditary optic neuropathy

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Neuroscience(all)
  • Psychiatry and Mental health

Cite this

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title = "α-tocopherol/lipid ratio in blood is decreased in patients with Leber's hereditary optic neuropathy and asymptomatic carriers of the 11778 mtDNA mutation",
abstract = "Objectives - Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease characterised by acute or subacute bilateral visual loss in young patients. The primary aetiological event is a mutation in the mitochondrial genome (mtDNA) affecting in most cases mtDNA-encoded subunits of the respiratory chain NADH: coenzyme Q oxidoreductase (complex I). The impaired function of complex I leads to a decline in mitochondrial energy production and enhances free radical generation. Methods - The concentrations of some non-enzymatic antioxidants (α-tocopherol, β-carotene, lycopene, glutathione, free sulphydryl groups) and the lipid peroxides in the blood of patients with LHON, carriers with homoplasmic DNA mutation at 11 778, and controls were investigated using high performance liquid chromatography and spectrophotometric methods to assess the function of their antioxidant defence systems. Results - The α-tocopherol/cholesterol+ triglyceride ratio was significantly reduced (p",
keywords = "α-tocopherol, Free radicals, Leber's hereditary optic neuropathy",
author = "P. Kliv{\'e}nyi and E. Karg and C. R{\'o}zsa and R. Horvath and S. Komoly and I. N{\'e}meth and S. T{\'u}ri and L. V{\'e}csei",
year = "2001",
month = "3",
doi = "10.1136/jnnp.70.3.359",
language = "English",
volume = "70",
pages = "359--362",
journal = "Journal of Neurology, Neurosurgery and Psychiatry",
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TY - JOUR

T1 - α-tocopherol/lipid ratio in blood is decreased in patients with Leber's hereditary optic neuropathy and asymptomatic carriers of the 11778 mtDNA mutation

AU - Klivényi, P.

AU - Karg, E.

AU - Rózsa, C.

AU - Horvath, R.

AU - Komoly, S.

AU - Németh, I.

AU - Túri, S.

AU - Vécsei, L.

PY - 2001/3

Y1 - 2001/3

N2 - Objectives - Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease characterised by acute or subacute bilateral visual loss in young patients. The primary aetiological event is a mutation in the mitochondrial genome (mtDNA) affecting in most cases mtDNA-encoded subunits of the respiratory chain NADH: coenzyme Q oxidoreductase (complex I). The impaired function of complex I leads to a decline in mitochondrial energy production and enhances free radical generation. Methods - The concentrations of some non-enzymatic antioxidants (α-tocopherol, β-carotene, lycopene, glutathione, free sulphydryl groups) and the lipid peroxides in the blood of patients with LHON, carriers with homoplasmic DNA mutation at 11 778, and controls were investigated using high performance liquid chromatography and spectrophotometric methods to assess the function of their antioxidant defence systems. Results - The α-tocopherol/cholesterol+ triglyceride ratio was significantly reduced (p

AB - Objectives - Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease characterised by acute or subacute bilateral visual loss in young patients. The primary aetiological event is a mutation in the mitochondrial genome (mtDNA) affecting in most cases mtDNA-encoded subunits of the respiratory chain NADH: coenzyme Q oxidoreductase (complex I). The impaired function of complex I leads to a decline in mitochondrial energy production and enhances free radical generation. Methods - The concentrations of some non-enzymatic antioxidants (α-tocopherol, β-carotene, lycopene, glutathione, free sulphydryl groups) and the lipid peroxides in the blood of patients with LHON, carriers with homoplasmic DNA mutation at 11 778, and controls were investigated using high performance liquid chromatography and spectrophotometric methods to assess the function of their antioxidant defence systems. Results - The α-tocopherol/cholesterol+ triglyceride ratio was significantly reduced (p

KW - α-tocopherol

KW - Free radicals

KW - Leber's hereditary optic neuropathy

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