肌萎缩侧索硬化视网膜损伤:潜在机制。

IF 3.1 Q1 OPHTHALMOLOGY
Eye and Brain Pub Date : 2021-05-12 eCollection Date: 2021-01-01 DOI:10.2147/EB.S299423
Vladislav O Soldatov, Michail S Kukharsky, Andrey E Belykh, Andrey M Sobolev, Alexey V Deykin
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引用次数: 11

摘要

肌萎缩侧索硬化症(ALS)是一种致命的神经退行性疾病,导致运动神经元功能逐渐丧失。虽然眼科症状目前不被认为是ALS的典型症状,但在许多患者中发现了视网膜病变,如变薄、轴突变性和包涵体。在死后的人体组织和动物模型中观察到的视网膜异常与ALS的脊髓变化相似。这些发现并不出人意料,因为视网膜与大脑有共同的个体发生关系,许多基因与神经变性和视网膜疾病都有关。实验研究表明,ALS会影响视网膜的许多“脆弱点”。聚体沉积、核蛋白输入受损、内质网应激、谷氨酸兴奋毒性、血管退化和线粒体功能障碍被认为是ALS运动神经元损伤的主要原因。在这里,我们展示了所有这些通路都可以像运动神经元一样影响视网膜细胞。此外,我们认为了解肌萎缩侧索硬化症中神经-眼相互作用的模式有助于该病的诊断和治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Retinal Damage in Amyotrophic Lateral Sclerosis: Underlying Mechanisms.

Retinal Damage in Amyotrophic Lateral Sclerosis: Underlying Mechanisms.

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease resulting in a gradual loss of motor neuron function. Although ophthalmic complaints are not presently considered a classic symptom of ALS, retinal changes such as thinning, axonal degeneration and inclusion bodies have been found in many patients. Retinal abnormalities observed in postmortem human tissues and animal models are similar to spinal cord changes in ALS. These findings are not dramatically unexpected because retina shares an ontogenetic relationship with the brain, and many genes are associated both with neurodegeneration and retinal diseases. Experimental studies have demonstrated that ALS affects many "vulnerable points" of the retina. Aggregate deposition, impaired nuclear protein import, endoplasmic reticulum stress, glutamate excitotoxicity, vascular regression, and mitochondrial dysfunction are factors suspected as being the main cause of motor neuron damage in ALS. Herein, we show that all of these pathways can affect retinal cells in the same way as motor neurons. Furthermore, we suppose that understanding the patterns of neuro-ophthalmic interaction in ALS can help in the diagnosis and treatment of this disease.

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来源期刊
Eye and Brain
Eye and Brain OPHTHALMOLOGY-
CiteScore
7.90
自引率
2.30%
发文量
12
审稿时长
16 weeks
期刊介绍: Eye and Brain is an international, peer-reviewed, open access journal focusing on basic research, clinical findings, and expert reviews in the field of visual science and neuro-ophthalmology. The journal’s unique focus is the link between two well-known visual centres, the eye and the brain, with an emphasis on the importance of such connections. All aspects of clinical and especially basic research on the visual system are addressed within the journal as well as significant future directions in vision research and therapeutic measures. This unique journal focuses on neurological aspects of vision – both physiological and pathological. The scope of the journal spans from the cornea to the associational visual cortex and all the visual centers in between. Topics range from basic biological mechanisms to therapeutic treatment, from simple organisms to humans, and utilizing techniques from molecular biology to behavior. The journal especially welcomes primary research articles or review papers that make the connection between the eye and the brain. Specific areas covered in the journal include: Physiology and pathophysiology of visual centers, Eye movement disorders and strabismus, Cellular, biochemical, and molecular features of the visual system, Structural and functional organization of the eye and of the visual cortex, Metabolic demands of the visual system, Diseases and disorders with neuro-ophthalmic manifestations, Clinical and experimental neuro-ophthalmology and visual system pathologies, Epidemiological studies.
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