Oxidative Stress in Chinese Patients with Leber's Hereditary Optic Neuropathy

The cellular mechanisms of Leber's hereditary optic neuropathy (LHON) are poorly understood and there is little information on the onset of blindness and neurological degeneration. Here we define the relationship between oxidative stress and LHON pathogenicity at the cellular level. Venous bloo...

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Bibliographic Details
Published in:Journal of international medical research 2008-05, Vol.36 (3), p.544-550
Main Authors: Wang, JY, Gu, YS, Wang, J, Tong, Y
Format: Article
Language:English
Subjects:
Adolescent
Adult
Asian Continental Ancestry Group
Base Sequence
Case-Control Studies
Child
China
DNA Mutational Analysis
DNA, Mitochondrial - genetics
Family
Female
Free Radicals - blood
Humans
Luminescence
Male
Molecular Sequence Data
Optic Atrophy, Hereditary, Leber - blood
Optic Atrophy, Hereditary, Leber - metabolism
Optic Disk - pathology
Oxidative Stress
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Summary:The cellular mechanisms of Leber's hereditary optic neuropathy (LHON) are poorly understood and there is little information on the onset of blindness and neurological degeneration. Here we define the relationship between oxidative stress and LHON pathogenicity at the cellular level. Venous blood was obtained from 14 patients with LHON, 21 asymptomatic maternal relatives and 30 normal individuals (controls). The level of free radicals in blood was assessed as luminol luminescence immediately and at 10 min after addition of phytohaemagglutinin. In LHON patients and their asymptomatic relatives, free radicals increased significantly immediately after adding phytohaemagglutinin compared with baseline and normal controls. After 10 min, however, there were no significant differences between and within the groups. These results suggest that the antioxidant capacity is reduced in the blood of patients with LHON and in asymptomatic relatives, and that oxidative stress plays a significant role in the pathogenesis of LHON.
ISSN:0300-0605
1473-2300
DOI:10.1177/147323000803600320