Mouse mtDNA mutant model of Leber hereditary optic neuropathy.
[No authors listed]
摘要
An animal model of Leber hereditary optic neuropathy (LHON) was produced by introducing the human optic atrophy mtDNA ND6 P25L mutation into the mouse. Mice with this mutation exhibited reduction in retinal function by elecroretinogram (ERG), age-related decline in central smaller caliber optic nerve fibers with sparing of larger peripheral fibers, neuronal accumulation of abnormal mitochondria, axonal swelling, and demyelination. Mitochondrial analysis revealed partial complex I and respiration defects and increased reactive oxygen species production, whereas synaptosome analysis revealed decreased complex I activity and increased but no diminution of ATP production. Thus, LHON pathophysiology may result from oxidative stress.
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基因功能
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原始数据
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文献解读
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