Pedersen Lotte B, Veland Iben R, Schrøder Jacob M, Christensen Søren T
Department of Biology, Section of Cell and Molecular Biology, University of Copenhagen, Copenhagen, Denmark.
Dev Dyn. 2008 Aug;237(8):1993-2006. doi: 10.1002/dvdy.21521.
Primary cilia are microtubule-based, hair-like sensory organelles present on the surface of most growth-arrested cells in our body. Recent research has demonstrated a crucial role for primary cilia in regulating vertebrate developmental pathways and tissue homeostasis, and defects in genes involved in primary cilia assembly or function have been associated with a panoply of disorders and diseases, including polycystic kidney disease, left-right asymmetry defects, hydrocephalus, and Bardet Biedl Syndrome. Here we provide an up-to-date review focused on the molecular mechanisms involved in the assembly of primary cilia in vertebrate cells. We present an overview of the early stages of the cilia assembly process, as well as a description of the intraflagellar transport (IFT) system. IFT is a highly conserved process required for assembly of almost all eukaryotic cilia and flagella, and much of our current knowledge about IFT is based on studies performed in Chlamydomonas and Caenorhabditis elegans. Therefore, our review of the IFT literature includes studies performed in these two model organisms. The role of several non-IFT proteins (e.g., centrosomal proteins) in the ciliary assembly process is also discussed.
初级纤毛是以微管为基础的毛发状感觉细胞器,存在于我们身体中大多数生长停滞细胞的表面。最近的研究表明,初级纤毛在调节脊椎动物发育途径和组织稳态方面起着关键作用,并且参与初级纤毛组装或功能的基因缺陷与一系列疾病相关,包括多囊肾病、左右不对称缺陷、脑积水和巴德-比德尔综合征。在此,我们提供一篇最新综述,重点关注脊椎动物细胞中初级纤毛组装所涉及的分子机制。我们概述了纤毛组装过程的早期阶段,并描述了鞭毛内运输(IFT)系统。IFT是几乎所有真核生物纤毛和鞭毛组装所需的高度保守过程,我们目前关于IFT的许多知识基于在衣藻和秀丽隐杆线虫中进行的研究。因此,我们对IFT文献的综述包括在这两种模式生物中进行的研究。还讨论了几种非IFT蛋白(如中心体蛋白)在纤毛组装过程中的作用。