高人一等（及以下）：蛋白切割在多囊蛋白运输和信号转导中的调控作用。
A cut above (and below): Protein cleavage in the regulation of polycystin trafficking and signaling.
机构信息
Broad Institute of MIT and Harvard, Massachusetts Institute of Technology, 415 Main Street, Cambridge, MA 02142, USA.
Department of Cellular and Molecular Physiology, Yale University School of Medicine, New Haven, CT 06520-8026, USA.
出版信息
Cell Signal. 2020 Aug;72:109634. doi: 10.1016/j.cellsig.2020.109634. Epub 2020 Apr 10.
The polycystin-1 and 2 proteins, encoded by the genes mutated in Autosomal Dominant Polycystic Kidney Disease, are connected to a large number of biological pathways. While the nature of these connections and their relevance to the primary functions of the polycystin proteins have yet to be fully elucidated, it is clear that many of them are mediated by or depend upon cleavage of the polycystin-1 protein. Cleavage of polycystin-1 at its G protein coupled receptor proteolytic site is an obligate step in the protein's maturation and in aspects of its trafficking. This cleavage may also serve to prime polycystin-1 to play a role as a non-canonical G protein coupled receptor. Cleavage of the cytoplasmic polycystin-1C terminal tail releases fragments that are able to enter the nucleus and the mitochondria and to influence their activities. Understanding the nature of these cleavages, their regulation and their consequences is likely to provide valuable insights into both the physiological functions served by the polycystin proteins and the pathological consequences of their absence.
多囊蛋白-1 和 2 蛋白由常染色体显性多囊肾病基因突变编码，与许多生物途径有关。虽然这些联系的性质及其与多囊蛋白蛋白主要功能的相关性尚未完全阐明，但很明显，其中许多是通过或依赖多囊蛋白-1 蛋白的切割来介导的。多囊蛋白-1 在其 G 蛋白偶联受体蛋白水解位点的切割是其成熟和运输的某些方面的必需步骤。这种切割也可能使多囊蛋白-1 充当非典型 G 蛋白偶联受体。细胞质多囊蛋白-1C 端尾部的切割释放能够进入细胞核和线粒体并影响其活性的片段。了解这些切割的性质、调节及其后果可能为多囊蛋白蛋白的生理功能及其缺失的病理后果提供有价值的见解。
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