International Review of Ophthalmology

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Research on mitochondrial damage in Leber hereditary optic neuropathy

ZHOU Bi-ting1, FANG Li-jun2, YAO Yi-hua1, ZHU Yi-hua1.   

  1. 1. Department of Ophthalmology, The First Affiliated Hospital of Fujian Medical University, Fuzhou 350108, China; 2. Department of Ophthalmology, Fujian Medical University Union Hospital, Fuzhou 350001, China
  • Received:2018-05-29 Online:2018-10-22 Published:2018-10-25
  • Contact: ZHU Yi-hua, Email: zhuyihua889@163.com
  • Supported by:

    National Natural Science Foundation of China(81270999); Natural Science Foundation of Fujian（2017J01292）

Abstract:

Leber hereditary optic neuropathy (LHON) is a maternally transmitted hereditary disease. Mitochondrial dysfunction and stress are involved in the degeneration and apoptosis of retinal ganglion cells (RGCs). The continuous innovation and improvement of the LHON models provide a good experimental basis for the study of the molecular mechanism of LHON. Among them, the ectopically expressed LHON mutant cells are the main research tools at this stage. Gene therapy is a breakthrough point in the treatment of LHON which has been tested in the clinic. (Int Rev Ophthalmol, 2018, 42:  302-306)