Leber hereditary optic neuropathy: respiratory chain dysfunction and degeneration of the optic nerve.

Abstract excerpt

Leber hereditary optic neuropathy (LHON) is an inherited form of bilateral optic atrophy in which the primary etiological event is a mutation in the mitochondrial genome. The optic neuropathy involves a loss of central vision due to degeneration of the retinal ganglion cells and optic nerve axons that subserve central vision. The primary mitochondrial mutation is necessary, but not sufficient, for manifestation...

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