Mitochondrial dysfunction underlying outer retinal diseases

作者：Lefevere Evy; Toft Kehler Anne Katrine; Vohra Rupali; Kolko Miriam; Moons Lieve; Van Hove Inge

来源：Mitochondrion, 2017, 36: 66-76.

DOI：10.1016/j.mito.2017.03.006

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

Dysfunction of photoreceptors, retinal pigment epithelium (RPE) or both contribute to the initiation and progression of several outer retinal disorders. Disrupted Muller 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 metabblically active photoreceptors/RPE are highly prone to these hallmarks of mitochondrial dysfunction, indicating that mitochondkia represent a weak link in the antioxidant defenses of outer retinal cells.

• 出版日期2017-9