From the *Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India; and †Laboratory for Molecular Reproduction and Genetics, Department of Anatomy, AIIMS, New Delhi, India.
Asia Pac J Ophthalmol (Phila). 2016 Jul-Aug;5(4):293-9. doi: 10.1097/APO.0000000000000203.
As a major source of cellular energy, mitochondria are critical for optimal ocular function. They are also essential for cell differentiation and survival. Mitochondrial mutations and oxidative damage to the mitochondrial DNA are important factors underlying the pathology of many ocular disorders. With increasing age, mitochondrial DNA damage accumulates and results in several eye diseases. It is evident that the mitochondrial genome is more susceptible to stress and damage than the nuclear genome, as it lacks histone protection, a nucleotide excision repair system, and recombination repair, and it is the source and target of free radicals. Accumulation of mitochondrial mutations beyond a certain threshold explains the marked variations in phenotypes seen in mitochondrial diseases and the molecular mechanisms related to the pathogenesis of several chronic disorders in the eye. This review details the structure and function of mitochondria and the mitochondrial genome along with the mitochondrial involvement in various neurodegenerative ophthalmic disorders.
线粒体作为细胞能量的主要来源，对最佳眼部功能至关重要。它们也是细胞分化和存活所必需的。线粒体突变和线粒体 DNA 的氧化损伤是许多眼部疾病病理的重要因素。随着年龄的增长，线粒体 DNA 损伤积累，导致多种眼部疾病。显然，与核基因组相比，线粒体基因组更容易受到压力和损伤的影响，因为它缺乏组蛋白保护、核苷酸切除修复系统和重组修复，并且是自由基的来源和靶点。线粒体突变的积累超过一定阈值可以解释线粒体疾病中表型的显著变化，以及与眼部几种慢性疾病发病机制相关的分子机制。本综述详细介绍了线粒体和线粒体基因组的结构和功能，以及线粒体在各种神经退行性眼科疾病中的作用。
