Mitochondrial dysfunction underlying outer retinal diseases

Mitochondrion. 2017 Sep:36:66-76. doi: 10.1016/j.mito.2017.03.006. Epub 2017 Mar 29.

Abstract

Dysfunction of photoreceptors, retinal pigment epithelium (RPE) or both contribute to the initiation and progression of several outer retinal disorders. Disrupted Müller glia function might additionally subsidize to these diseases. Mitochondrial malfunctioning is importantly associated with outer retina pathologies, which can be classified as primary and secondary mitochondrial disorders. This review highlights the importance of oxidative stress and mitochondrial DNA damage, underlying outer retinal disorders. Indeed, the metabolically active photoreceptors/RPE are highly prone to these hallmarks of mitochondrial dysfunction, indicating that mitochondria represent a weak link in the antioxidant defenses of outer retinal cells.

Keywords: Age-related Macular Degeneration; Kearns-Sayre Syndrome; Mitochondria; Neuropathy Ataxia Retinitis Pigmentosa Syndrome; Photoreceptors; Retinal pigment epithelium.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • DNA Damage
  • DNA, Mitochondrial / genetics
  • Ependymoglial Cells / pathology
  • Humans
  • Mitochondria / pathology*
  • Oxidative Stress
  • Photoreceptor Cells, Vertebrate / pathology
  • Retinal Diseases / pathology*
  • Retinal Diseases / physiopathology*
  • Retinal Pigment Epithelium / pathology

Substances

  • DNA, Mitochondrial