鞭毛内运输在光感受器感觉纤毛中的作用。

The Role of Intraflagellar Transport in the Photoreceptor Sensory Cilium.

作者信息

Taub Daniel G, Liu Qin

机构信息

Ocular Genomics Institute, and Berman-Gund Laboratory for the Study of Retinal Degenerations, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St., 5th Floor, 563C, 02114, Boston, MA, USA.

出版信息

Adv Exp Med Biol. 2016;854:627-33. doi: 10.1007/978-3-319-17121-0_83.

DOI:10.1007/978-3-319-17121-0_83

PMID:26427468

Abstract

The photoreceptor is a complex specialized cell in which a major component responsible for visual transduction is the photoreceptor sensory cilium (PSC). Building and maintenance of the PSC requires the transport of large proteins along microtubules that extend from the inner segments to the outer segments. A key process, termed intraflagellar transport (IFT), has been recognized as an essential phenomenon for photoreceptor development and maintenance, and exciting new studies have highlighted its importance in retinal and cilia related diseases. This review focuses on the important roles of IFT players, including motor proteins, IFT proteins, and photoreceptor-specific cargos in photoreceptor sensory cilium. In addition, specific IFT components that are involved in inherited human diseases are discussed.

摘要

光感受器是一种复杂的特化细胞，其中负责视觉转导的主要成分是光感受器感觉纤毛（PSC）。PSC的构建和维持需要大蛋白沿着从内段延伸到外段的微管进行运输。一个关键过程，称为鞭毛内运输（IFT），已被认为是光感受器发育和维持的基本现象，并且令人兴奋的新研究突出了其在视网膜和纤毛相关疾病中的重要性。本综述重点关注IFT相关蛋白，包括驱动蛋白、IFT蛋白和光感受器特异性货物在光感受器感觉纤毛中的重要作用。此外，还讨论了与人类遗传性疾病相关的特定IFT成分。