SCI TOP 10

当前位置:首页>SCI TOP 10

视杆细胞形态和疾病的结构和分子学基础

Wensel TG, Zhang Z, Anastassov IA, et al.

期刊名称:Progress in Retinal and Eye Research

卷期:2016年第55卷

关键词:
形态 细胞 分子

摘要

视杆细胞具有一种非常特殊的结构,使其具有检测单个光子的独特功能。视杆的结构特征和高度放大光检测所需的代谢过程似乎使得视杆细胞对结构和代谢缺陷特别敏感,因此大量基因缺陷主要与杆细胞死亡相关,并产生致盲性视网膜营养不良。视杆细胞结构,特别是被称为外段的感觉纤毛结构,已是多年结构,生物化学和遗传分析的主题,但视杆营养不良中视杆细胞的形态变化和细胞死亡的分子基础仍然了解不足。成像技术的最新发展,如低温电子断层扫描和超分辨荧光显微镜,基因测序技术和基因编辑技术,正在迅速为我们理解这些问题带来新突破。我们将介绍我们目前对这些问题的理解,强调不确定性和有争议的领域,以及最近有关新见解的发现。还将提出来自新兴成像技术领域的结构数据的示例。

The rod cell has an extraordinarily specialized structure that allows it to carry out its unique function of detecting individual photons of light. Both the structural features of the rod and the metabolic processes required for highly amplified light detection seem to have rendered the rod especially sensitive to structural and metabolic defects, so that a large number of gene defects are primarily associated with rod cell death and give rise to blinding retinal dystrophies. The structures of the rod, especially those of the sensory cilium known as the outer segment, have been the subject of structural, biochemical, and genetic analysis for many years, but the molecular bases for rod morphogenesis and for cell death in rod dystrophies are still poorly understood. Recent developments in imaging technology, such as cryo-electron tomography and super-resolution fluorescence microscopy, in gene sequencing technology, and in gene editing technology are rapidly leading to new breakthroughs in our understanding of these questions. A summary is presented of our current understanding of selected aspects of these questions, highlighting areas of uncertainty and contention as well as recent discoveries that provide new insights. Examples of structural data from emerging imaging technologies are presented.


点击下载

CopyRight 2016  兴齐100版权所有 京ICP证060955号
技术支持:示剑网络   http://100.sinqi.com/