原发性纤毛在纤毛疾病中的再思考:纤毛蛋白的细胞外和纤毛功能是否集中在极性主题上?
Primary Cilia Reconsidered in the Context of Ciliopathies: Extraciliary and Ciliary Functions of Cilia Proteins Converge on a Polarity theme?
机构信息
Department of Neuroscience and Experimental Therapeutics, Albany Medical College, 47 New Scotland Avenue, MC-136, Albany, NY, 12208, USA.
Department of Neurology, Albany Medical College, Albany, NY, 12208, USA.
出版信息
Bioessays. 2018 Aug;40(8):e1700132. doi: 10.1002/bies.201700132. Epub 2018 Jun 8.
Once dismissed as vestigial organelles, primary cilia have garnered the interest of scientists, given their importance in development/signaling, and for their implication in a new disease category known as ciliopathies. However, many, if not all, "cilia" proteins also have locations/functions outside of the primary cilium. These extraciliary functions can complicate the interpretation of a particular ciliopathy phenotype: it may be a result of defects at the cilium and/or at extraciliary locations, and it could be broadly related to a unifying cellular process for these proteins, such as polarity. Assembly of a cilium has many similarities to the development of other polarized structures. This evolutionarily preserved process for the assembly of polarized cell structures offers a perspective on how the cilium may have evolved. We hypothesize that cilia proteins are critical for cell polarity, and that core polarity proteins may have been specialized to form various cellular protrusions, including primary cilia.
曾经被认为是退化的细胞器,初级纤毛因其在发育/信号转导中的重要性以及在一种新的疾病类别——纤毛病中的作用而引起了科学家的兴趣。然而,许多(如果不是全部的话)“纤毛”蛋白也有不在初级纤毛中的位置/功能。这些细胞外功能可能会使特定纤毛病表型的解释变得复杂:它可能是纤毛和/或细胞外位置缺陷的结果,并且可能与这些蛋白质的统一细胞过程广泛相关,例如极性。纤毛的组装与其他极化结构的发育有许多相似之处。这种对极化细胞结构组装的进化保守过程为纤毛的进化提供了一个视角。我们假设纤毛蛋白对于细胞极性至关重要,并且核心极性蛋白可能已经专门用于形成各种细胞突起,包括初级纤毛。