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发育和疾病中的平面细胞极性

Planar cell polarity in development and disease.

作者信息

Butler Mitchell T, Wallingford John B

机构信息

Department of Molecular Biosciences, Patterson Labs, 2401 Speedway, The University of Texas at Austin, Austin, Texas 78712, USA.

出版信息

Nat Rev Mol Cell Biol. 2017 Jun;18(6):375-388. doi: 10.1038/nrm.2017.11. Epub 2017 Mar 15.

Abstract

Planar cell polarity (PCP) is an essential feature of animal tissues, whereby distinct polarity is established within the plane of a cell sheet. Tissue-wide establishment of PCP is driven by multiple global cues, including gradients of gene expression, gradients of secreted WNT ligands and anisotropic tissue strain. These cues guide the dynamic, subcellular enrichment of PCP proteins, which can self-assemble into mutually exclusive complexes at opposite sides of a cell. Endocytosis, endosomal trafficking and degradation dynamics of PCP components further regulate planar tissue patterning. This polarization propagates throughout the whole tissue, providing a polarity axis that governs collective morphogenetic events such as the orientation of subcellular structures and cell rearrangements. Reflecting the necessity of polarized cellular behaviours for proper development and function of diverse organs, defects in PCP have been implicated in human pathologies, most notably in severe birth defects.

摘要

平面细胞极性(PCP)是动物组织的一个基本特征,通过它可以在细胞层平面内建立不同的极性。PCP在整个组织范围内的建立是由多种全局信号驱动的,包括基因表达梯度、分泌的WNT配体梯度和各向异性组织应变。这些信号引导PCP蛋白在亚细胞水平上动态富集,PCP蛋白可以在细胞的相对两侧自组装成相互排斥的复合物。PCP组分的内吞作用、内体运输和降解动力学进一步调节平面组织的图案形成。这种极化在整个组织中传播,提供一个极性轴,该极性轴控制集体形态发生事件,如亚细胞结构的定向和细胞重排。由于极化细胞行为对于多种器官的正常发育和功能是必需的,PCP缺陷已被认为与人类疾病有关,最显著的是严重出生缺陷。

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