title: Mitochondrial dysfunction underlying outer retinal diseases.
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.
authors.affiliation: Neural Circuit Development and Regeneration Research Group, Department of Biology, KU Leuven, Leuven, Belgium.
authors.forename: Evy
authors.initials: E
authors.lastname: Lefevere
authors.affiliation: Department of Neuroscience and Pharmacology, Faculty of Health Sciences, Center for Healthy Aging, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Anne Katrine
authors.initials: AK
authors.lastname: Toft-Kehler
authors.affiliation: Department of Neuroscience and Pharmacology, Faculty of Health Sciences, Center for Healthy Aging, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Rupali
authors.initials: R
authors.lastname: Vohra
authors.affiliation: Department of Neuroscience and Pharmacology, Faculty of Health Sciences, Center for Healthy Aging, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Miriam
authors.initials: M
authors.lastname: Kolko
authors.affiliation: Neural Circuit Development and Regeneration Research Group, Department of Biology, KU Leuven, Leuven, Belgium. Electronic address: Lieve.moons@kuleuven.be.
authors.forename: Lieve
authors.initials: L
authors.lastname: Moons
authors.affiliation: Neural Circuit Development and Regeneration Research Group, Department of Biology, KU Leuven, Leuven, Belgium.
authors.forename: Inge
authors.initials: I
authors.lastname: Van Hove
mesh_terms: D004249:DNA Damage; D004272:DNA, Mitochondrial / Q000235:genetics; D063928:Ependymoglial Cells / Q000473:pathology; D006801:Humans; D008928:Mitochondria / Q000473:pathology*; D018384:Oxidative Stress; D020419:Photoreceptor Cells, Vertebrate / Q000473:pathology; D012164:Retinal Diseases / Q000473:pathology* / Q000503:physiopathology*; D055213:Retinal Pigment Epithelium / Q000473:pathology
chemical_list: D004272:DNA, Mitochondrial
keywords: Age-related Macular Degeneration; Kearns-Sayre Syndrome; Mitochondria; Neuropathy Ataxia Retinitis Pigmentosa Syndrome; Photoreceptors; Retinal pigment epithelium