Author:

Keywords:

Science & Technology, Life Sciences & Biomedicine, Cell Biology, Genetics & Heredity, Mitochondria, Photoreceptors, Retinal pigment epithelium, Age-related Macular Degeneration, Keams-Sayre Syndrome, Neuropathy Ataxia Retinitis Pigmentosa, Syndrome, KEARNS-SAYRE SYNDROME, CONE CELL-DEATH, HEREDITARY SPASTIC PARAPLEGIA, NEUROGENIC MUSCLE WEAKNESS, PIGMENT EPITHELIAL-CELLS, COMPLEMENT FACTOR-H, OXIDATIVE STRESS, DNA DAMAGE, RETINITIS-PIGMENTOSA, MULLER CELLS, Kearns-Sayre Syndrome, Neuropathy Ataxia Retinitis Pigmentosa Syndrome, DNA Damage, DNA, Mitochondrial, Ependymoglial Cells, Humans, Oxidative Stress, Photoreceptor Cells, Vertebrate, Retinal Diseases, Retinal Pigment Epithelium, 0604 Genetics, Biochemistry & Molecular Biology, 3101 Biochemistry and cell biology, 3105 Genetics

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.