Adler Paul N, Wallingford John B
Departments of Biology and Cell Biology, University of Virginia School of Medicine, Charlottesville, VA 22908, USA.
Department of Molecular Biosciences, University of Texas at Austin, Austin, TX 78712, USA.
Trends Cell Biol. 2017 May;27(5):379-390. doi: 10.1016/j.tcb.2016.12.001. Epub 2017 Jan 30.
Why some genes are more popular than others remains an open question, but one example of this phenomenon involves the genes controlling planar cell polarity (PCP), the polarization of cells within a plane of a tissue. Indeed, the so-called 'core' PCP genes such as dishevelled, frizzled, and prickle have been extensively studied both in animal models and by human genetics. By contrast, other genes that influence PCP signaling have received far less attention. Among the latter are inturned, fuzzy, and fritz, but recent work should bring these once obscure regulators into the limelight. We provide here a brief history of planar polarity effector (PPE) and CPLANE (ciliogenesis and planar polarity effector) proteins, discuss recent advances in understanding their molecular mechanisms of action, and describe their roles in human disease.
为什么有些基因比其他基因更受关注仍是一个悬而未决的问题，但这种现象的一个例子涉及控制平面细胞极性（PCP）的基因，即组织平面内细胞的极化。事实上，诸如蓬乱蛋白、卷曲蛋白和刺状蛋白等所谓的“核心”PCP基因已经在动物模型和人类遗传学中得到了广泛研究。相比之下，其他影响PCP信号传导的基因受到的关注要少得多。后者包括内翻蛋白、模糊蛋白和弗里茨蛋白，但最近的研究应该会让这些曾经默默无闻的调节因子成为焦点。我们在此简要介绍平面极性效应器（PPE）和CPLANE（纤毛发生和平面极性效应器）蛋白的历史，讨论在理解其分子作用机制方面的最新进展，并描述它们在人类疾病中的作用。
