[Medical treatments in Leber's hereditary optic neuropathy].

Abstract excerpt

Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease caused by a mutation of mitochondrial DNA. LHON targets retinal ganglion cells (RGC), whose axons form the optic nerve. The mutation that leads to LHON is silent until an unknown trigger causes dysfunction of complex I in the mitochondria of RGC. This results in discontinuation of RGC energy production and, eventually, RGC apoptosis....

Topics

• Humans
• Optic Atrophy, Hereditary, Leber
• Retinal Ganglion Cells
• DNA, Mitochondrial
• Optic Nerve
• Mutation