Suppr超能文献

先天性和年龄相关性白内障的突变及机制

Mutations and mechanisms in congenital and age-related cataracts.

作者信息

Shiels Alan, Hejtmancik J Fielding

机构信息

Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St. Louis, MO 63110, USA.

Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, MD 20892-1860, USA.

出版信息

Exp Eye Res. 2017 Mar;156:95-102. doi: 10.1016/j.exer.2016.06.011. Epub 2016 Jun 19.

Abstract

The crystalline lens plays an important role in the refractive vision of vertebrates by facilitating variable fine focusing of light onto the retina. Loss of lens transparency, or cataract, is a frequently acquired cause of visual impairment in adults and may also present during childhood. Genetic studies have identified mutations in over 30 causative genes for congenital or other early-onset forms of cataract as well as several gene variants associated with age-related cataract. However, the pathogenic mechanisms resulting from genetic determinants of cataract are only just beginning to be understood. Here, we briefly summarize current concepts pointing to differences in the molecular mechanisms underlying congenital and age-related forms of cataract.

摘要

晶状体通过促进光线可变的精细聚焦到视网膜上,在脊椎动物的屈光视觉中发挥重要作用。晶状体透明度丧失,即白内障,是成人常见的后天性视力损害原因,也可能在儿童期出现。遗传学研究已经确定了30多种先天性或其他早发性白内障致病基因的突变,以及几种与年龄相关性白内障相关的基因变异。然而,由白内障遗传决定因素导致的致病机制才刚刚开始被理解。在这里,我们简要总结当前的概念,指出先天性和年龄相关性白内障潜在分子机制的差异。

相似文献

1
Mutations and mechanisms in congenital and age-related cataracts.先天性和年龄相关性白内障的突变及机制
Exp Eye Res. 2017 Mar;156:95-102. doi: 10.1016/j.exer.2016.06.011. Epub 2016 Jun 19.
2
Inherited cataracts: Genetic mechanisms and pathways new and old.遗传性白内障:新旧遗传机制和途径。
Exp Eye Res. 2021 Aug;209:108662. doi: 10.1016/j.exer.2021.108662. Epub 2021 Jun 12.
6
[Research progress in relative crystallin genes of congenital cataract].[先天性白内障相关晶状体蛋白基因的研究进展]
Zhonghua Yan Ke Za Zhi. 2016 Feb;52(2):141-9. doi: 10.3760/cma.j.issn.0412-4081.2016.02.016.
7
Differential role of arginine mutations on the structure and functions of α-crystallin.精氨酸突变对α-晶状体蛋白结构和功能的不同作用。
Biochim Biophys Acta. 2016 Jan;1860(1 Pt B):199-210. doi: 10.1016/j.bbagen.2015.06.004. Epub 2015 Jun 14.
8
Molecular genetics of age-related cataract.年龄相关性白内障的分子遗传学
Exp Eye Res. 2004 Jul;79(1):3-9. doi: 10.1016/j.exer.2004.03.014.
9
Developmental genetics in ophthalmology.眼科发育遗传学
Ophthalmic Genet. 2003 Mar;24(1):1-33. doi: 10.1076/opge.24.1.1.13888.

引用本文的文献

3

本文引用的文献

5
The etiology of human age-related cataract. Proteins don't last forever.人类年龄相关性白内障的病因。蛋白质不会永远存在。
Biochim Biophys Acta. 2016 Jan;1860(1 Pt B):192-8. doi: 10.1016/j.bbagen.2015.08.016. Epub 2015 Aug 28.
6
Lanosterol reverses protein aggregation in cataracts.羊毛甾醇可逆转白内障中的蛋白质聚集。
Nature. 2015 Jul 30;523(7562):607-11. doi: 10.1038/nature14650. Epub 2015 Jul 22.
8
Structure and function of α-crystallins: Traversing from in vitro to in vivo.α-晶状体蛋白的结构与功能:从体外到体内的研究进展
Biochim Biophys Acta. 2016 Jan;1860(1 Pt B):149-66. doi: 10.1016/j.bbagen.2015.06.008. Epub 2015 Jun 25.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验