Adaptive changes in the visual cortex after photoreceptor degeneration in retinitis pigmentosa.

IF 2.5 4区 生物学 Q3 CELL BIOLOGY
Juan R Martinez-Galan, Elena Caminos
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引用次数: 0

Abstract

Retinitis pigmentosa (RP) is a group of hereditary disorders that cause progressive retinal degeneration, affecting the rods and, subsequently, the cones, which results in progressive vision loss. RP is genetically heterogeneous and is inherited in an autosomal dominant, autosomal recessive, X-linked, or sporadic non-Mendelian manner. The recent advancements in repairing damaged retinas highlight the necessity of understanding the impact of photoreceptor degeneration on the visual cortex. This is because functional vision may not be adequately restored if this region is significantly impaired prior to treatment. In the present review, we have analyzed the rodent models of RP that have been most frequently used and the physiological and morphological changes occurring in both humans and rodents with this disorder. Following visually evoked stimulation, the processing of visual information in the primary visual cortex (V1) of individuals with RP is altered due to modifications in the transduction of the signal originating in the degenerated retina. Moreover, alterations in the intrinsic electrophysiological properties of cortical neurons and neural circuits have also been documented. Finally, several neurochemical and/or morphological changes are observed in synaptic structures associated with pyramidal neurons and in select inhibitory interneurons. Nevertheless, despite the physiological and morphological changes that have been described, the impact of RP on the visual cortex does not inevitably result in irreversible damage, as the alterations do not appear to be particularly severe. Brain plasticity is more restricted in adults; however, remodeling of the visual cortex in mice and humans is possible, which encourages further work on therapies capable of partially restoring the lost visual function.

色素性视网膜炎光感受器变性后视皮层的适应性变化。
色素性视网膜炎(RP)是一组遗传性疾病,引起进行性视网膜变性,影响视杆细胞,随后影响视锥细胞,导致进行性视力丧失。RP是遗传异质性的,以常染色体显性、常染色体隐性、x连锁或散发的非孟德尔方式遗传。近年来在视网膜损伤修复方面的进展突出了理解视觉皮层中感光体退化的影响的必要性。这是因为如果该区域在治疗前严重受损,功能视力可能无法充分恢复。在本文中,我们分析了RP最常用的啮齿动物模型,以及该疾病在人类和啮齿动物中发生的生理和形态学变化。在视觉诱发刺激后,RP个体的初级视觉皮层(V1)的视觉信息处理由于源自退化视网膜的信号转导的改变而发生改变。此外,皮层神经元和神经回路的内在电生理特性的改变也有文献记载。最后,在锥体神经元和选择性抑制性中间神经元相关的突触结构中观察到一些神经化学和/或形态学变化。然而,尽管已经描述了生理和形态上的变化,RP对视觉皮层的影响并不必然导致不可逆的损伤,因为这种改变似乎并不特别严重。成年人的大脑可塑性更受限制;然而,小鼠和人类视觉皮层的重塑是可能的,这鼓励了进一步研究能够部分恢复失去的视觉功能的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Histology and histopathology
Histology and histopathology 生物-病理学
CiteScore
3.90
自引率
0.00%
发文量
232
审稿时长
2 months
期刊介绍: HISTOLOGY AND HISTOPATHOLOGY is a peer-reviewed international journal, the purpose of which is to publish original and review articles in all fields of the microscopical morphology, cell biology and tissue engineering; high quality is the overall consideration. Its format is the standard international size of 21 x 27.7 cm. One volume is published every year (more than 1,300 pages, approximately 90 original works and 40 reviews). Each volume consists of 12 numbers published monthly online. The printed version of the journal includes 4 books every year; each of them compiles 3 numbers previously published online.
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