Leber Hereditary Optic Neuropathy: How Do Mitochondrial DNA Mutations Cause Degeneration of the Optic Nerve?

@article{Howell1997LeberHO,
  title={Leber Hereditary Optic Neuropathy: How Do Mitochondrial DNA Mutations Cause Degeneration of the Optic Nerve?},
  author={Neil Howell},
  journal={Journal of Bioenergetics and Biomembranes},
  year={1997},
  volume={29},
  pages={165-173}
}
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 development of the optic neuropathy, and secondary genetic and/or epigenetic risk factors must also… CONTINUE READING

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