多囊肾病中的睫状肌功能障碍：一种具有极化潜力的新兴模型。
Ciliary dysfunction in polycystic kidney disease: an emerging model with polarizing potential.
作者信息
Kolb Robert J, Nauli Surya M
机构信息
Department of Pediatrics, Medical University of South Carolina, Charleston, SC 29425, USA.
出版信息
Front Biosci. 2008 May 1;13:4451-66. doi: 10.2741/3016.
Abstract
The majority of different cell types in the human body have a cilium, a thin rod-like structure of uniquely arranged microtubules that are encapsulated by the surface plasma membrane. The cilium originates from a basal body, a mature centriole that has migrated and docked to the cell surface. The non-motile cilia are microtubule-based organelles that are generally considered sensory structures. The purpose of this review is to discuss the practicality of the ciliary hypothesis as a unifying concept for polycystic kidney disease and to review current literature in the field of cilium biology, as it relates to mechanosensation and planar cell polarity. The polycystins and fibrocystin localization at the cilium and other subcellular localizations are discussed, followed by a hypothetical model for the cilium's role in mechanosensing, planar cell polarity, and cystogenesis.
摘要
人体中大多数不同类型的细胞都有一根纤毛，这是一种由独特排列的微管组成的细杆状结构，被表面质膜包裹。纤毛起源于基体，即一个迁移并对接至细胞表面的成熟中心粒。非运动性纤毛是以微管为基础的细胞器，通常被认为是感觉结构。本综述的目的是讨论纤毛假说作为多囊肾病统一概念的实用性，并回顾纤毛生物学领域中与机械感觉和平面细胞极性相关的当前文献。文中讨论了多囊蛋白和纤维囊蛋白在纤毛及其他亚细胞定位中的情况，随后提出了一个关于纤毛在机械感觉、平面细胞极性和囊肿形成中作用的假说模型。
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