Department of Molecular and Biomedical Pharmacology, College of Medicine, University of Kentucky, Lexington, KY, USA.
Ophthalmic Res. 2010;44(3):179-90. doi: 10.1159/000316480. Epub 2010 Sep 9.
Mitochondria are critical for ocular function as they represent the major source of a cell's supply of energy and play an important role in cell differentiation and survival. Mitochondrial dysfunction can occur as a result of inherited mitochondrial mutations (e.g. Leber's hereditary optic neuropathy and chronic progressive external ophthalmoplegia) or stochastic oxidative damage which leads to cumulative mitochondrial damage and is an important factor in age-related disorders (e.g. age-related macular degeneration, cataract and diabetic retinopathy). Mitochondrial DNA (mtDNA) instability is an important factor in mitochondrial impairment culminating in age-related changes and pathology, and in all regions of the eye mtDNA damage is increased as a consequence of aging and age-related disease. It is now apparent that the mitochondrial genome is a weak link in the defenses of ocular cells since it is susceptible to oxidative damage and it lacks some of the systems that protect the nuclear genome, such as nucleotide excision repair. Accumulation of mitochondrial mutations leads to cellular dysfunction and increased susceptibility to adverse events which contribute to the pathogenesis of numerous sporadic and chronic disorders in the eye.
线粒体对于眼部功能至关重要，因为它们是细胞能量供应的主要来源，并在细胞分化和存活中发挥重要作用。线粒体功能障碍可能是由于遗传的线粒体突变（例如莱伯遗传性视神经病变和慢性进行性外眼肌麻痹）或随机的氧化损伤引起的，这会导致线粒体累积损伤，是与年龄相关疾病（例如年龄相关性黄斑变性、白内障和糖尿病性视网膜病变）的重要因素。线粒体 DNA（mtDNA）不稳定性是导致与年龄相关的变化和病理学相关的线粒体损伤的重要因素，在眼睛的所有区域，由于衰老和与年龄相关的疾病，mtDNA 损伤都会增加。现在很明显，线粒体基因组是眼部细胞防御中的一个薄弱环节，因为它容易受到氧化损伤，并且缺乏一些保护核基因组的系统，例如核苷酸切除修复。线粒体突变的积累导致细胞功能障碍和对不利事件的易感性增加，这有助于许多散发性和慢性眼部疾病的发病机制。
