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首页> 外文期刊>Neurochemistry International: The International Journal for the Rapid Publication of Critical Reviews, Preliminary and Original Research Communications in Neurochemistry >Optic nerve degeneration and mitochondrial dysfunction: genetic and acquired optic neuropathies.
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Optic nerve degeneration and mitochondrial dysfunction: genetic and acquired optic neuropathies.

机译：视神经变性和线粒体功能障碍：遗传性和获得性视神经病变。

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摘要

Selective degeneration of the smallest fibers (papillo-macular bundle) of the human optic nerve occurs in a large number of optic neuropathies characterized primarily by loss of central vision. The pathophysiology that underlies this peculiar pattern of cell involvement probably reflects different forms of genetic and acquired mitochondrial dysfunction.Maternally inherited Leber's hereditary optic neuropathy (LHON), dominant optic atrophy (Kjer disease), the optic atrophy of Leigh's syndrome, Friedreich ataxia and a variety of other conditions are examples of inherited mitochondrial disorders with different etiologies. Tobacco-alcohol amblyopia (TAA), the Cuban epidemic of optic neuropathy (CEON) and other dietary (Vitamins B, folate deficiencies) optic neuropathies, as well as toxic optic neuropathies such as due to chloramphenicol, ethambutol, or more rarely to carbon monoxide, methanol and cyanide are probably all related forms of acquired mitochondrial dysfunction.Biochemical and cellular studies in LHON point to a partial defect of respiratory chain function that may generate either an ATP synthesis defect and/or a chronic increase of oxidative stress. Histopathological studies in LHON cases and a rat model mimicking CEON revealed a selective loss of retinal ganglion cells (RGCs) and the corresponding axons, particularly in the temporal-central part of the optic nerve. Anatomical peculiarities of optic nerve axons, such as the asymmetric pattern of myelination, may have functional implications on energy dependence and distribution of mitochondrial populations in the different sections of the nerve. Histological evidence suggests impaired axonal transport of mitochondria in LHON and in the CEON-like rat model, indicating a possible common pathophysiology for this category of optic neuropathies. Histological evidence of myelin pathology in LHON also suggests a role for oxidative stress, possibly affecting the oligodendrocytes of the optic nerves.
机译：人类视神经的最小纤维（乳头状黄斑束）的选择性变性发生在大量以中央视力丧失为特征的视神经病变中。这种特殊的细胞参与模式的病理生理学可能反映了遗传和后天的线粒体功能障碍的不同形式。其他各种疾病是具有不同病因的遗传性线粒体疾病的例子。烟草酒精性弱视（TAA），古巴流行性视神经病（CEON）和其他饮食性（维生素B，叶酸缺乏）视神经病，以及中毒性视神经病，例如由于氯霉素，乙胺丁醇或更常见于一氧化碳甲醇和氰化物可能都是获得性线粒体功能障碍的相关形式。LHON的生化和细胞研究表明，呼吸链功能的部分缺陷可能导致ATP合成缺陷和/或氧化应激的慢性增加。在LHON病例和模仿CEON的大鼠模型中的组织病理学研究显示，视网膜神经节细胞（RGC）和相应的轴突选择性丢失，尤其是在视神经的颞中部。视神经轴突的解剖学特征（例如髓鞘的不对称模式）可能对能量依赖性和神经不同部分中线粒体种群的分布具有功能上的影响。组织学证据表明，在LHON和CEON样大鼠模型中，线粒体的轴突运输受损，这表明这类视神经病可能存在常见的病理生理学。 LHON中髓鞘病理的组织学证据也表明氧化应激的作用，可能影响视神经的少突胶质细胞。

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