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遗传性疾病与神经眼科的最新进展。

Recent advances in hereditary disease and neuro-ophthalmology.

作者信息

Mackey D A

机构信息

University of Melbourne, Royal Victorian Eye and Ear Hospital, East Melbourne, Australia.

出版信息

Curr Opin Ophthalmol. 1995 Dec;6(6):48-53. doi: 10.1097/00055735-199512000-00008.

Abstract

Recent advances in genetics tend to center on the discoveries of molecular biology. A disease is first linked to a region on a chromosome, a gene is later cloned, or a candidate gene identified, point mutations described, phenotype-genotype correlations made, and rationales for treatment proposed. Several neuro-ophthalmological diseases have recently been studied in this way; including Leber's hereditary optic neuropathy and other mitochondrial diseases, autosomal dominant (Kjer) optic atrophy, Wolfram syndrome, or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), Usher syndrome, neurofibromatosis types I and II, and two disorders of the paired box genes: aniridia and Waardenburg's syndrome. Apart from molecular biology there are still some new disease entities being described and new inheritance patterns identified for some syndromes, such as periodic alternating nystagmus.

摘要

遗传学的最新进展往往集中在分子生物学的发现上。一种疾病首先与染色体上的一个区域相关联,随后克隆出一个基因,或者鉴定出一个候选基因,描述点突变,建立表型与基因型的关联,并提出治疗的基本原理。最近,几种神经眼科疾病就是通过这种方式进行研究的;包括Leber遗传性视神经病变和其他线粒体疾病、常染色体显性(凯尔型)视神经萎缩、Wolfram综合征或尿崩症、糖尿病、视神经萎缩和耳聋综合征(DIDMOAD)、Usher综合征、I型和II型神经纤维瘤病,以及两种配对盒基因疾病:无虹膜和瓦登伯革氏综合征。除了分子生物学之外,仍有一些新的疾病实体被描述出来,并且为一些综合征确定了新的遗传模式,比如周期性交替性眼球震颤。

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