Mitochondrial dysfunction underlying outer retinal diseases

Evy Lefevere, Anne Katrine Toft-Kehler, Rupali Vohra, Miriam Kolko, Lieve Moons*, Inge Van Hove

*Corresponding author for this work
42 Citations (Scopus)

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.

Original languageEnglish
JournalMitochondrion
Volume36
Pages (from-to)66-76
ISSN1567-7249
DOIs
Publication statusPublished - Sept 2017

Keywords

  • Age-related Macular Degeneration
  • Kearns-Sayre Syndrome
  • Mitochondria
  • Neuropathy Ataxia Retinitis Pigmentosa Syndrome
  • Photoreceptors
  • Retinal pigment epithelium

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