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夜盲症再探讨:从人类到分子。普罗克特讲座。

Night blindness revisited: from man to molecules. Proctor lecture.

作者信息

Ripps H

出版信息

Invest Ophthalmol Vis Sci. 1982 Nov;23(5):588-609.

PMID:6127328
Abstract

Night blindness is a frequent concomitant of retinal disorders, many of which are of genetic origin. Through the use of quantitative noninvasive test procedures it has been possible to study patients with these hereditary conditions and to show that the visual abnormalities often result from defects in the functional properties of the rod photoreceptors. More important, the uniqueness of the functional disturbance in the various types of night-blinding disorders suggests that each involves a specific aspect of the rod's internal machinery, i.e., the molecular processes devoted to transduction, intercellular communication, and the renewal of cellular components. Knowledge gained from the study of these clinical entities and from the investigation of experimental animals regarding the cellular events involved in these vital processes have enabled us to formulate tentative hypothesis as to the molecular bases of the hereditary defects.

摘要

夜盲症是视网膜疾病常见的伴随症状,其中许多是遗传性的。通过使用定量非侵入性检测程序,得以研究患有这些遗传性疾病的患者,并表明视觉异常通常是由视杆光感受器功能特性缺陷所致。更重要的是,各种类型夜盲症功能障碍的独特性表明,每种类型都涉及视杆细胞内部机制的特定方面,即致力于转导、细胞间通讯以及细胞成分更新的分子过程。从对这些临床实体的研究以及对实验动物参与这些重要过程的细胞事件的调查中获得的知识,使我们能够就遗传性缺陷的分子基础提出初步假设。

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