Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA.
J Cell Biol. 2010 Jan 25;188(2):181-90. doi: 10.1083/jcb.200908114. Epub 2009 Dec 1.
Pericentrin is an integral component of the centrosome that serves as a multifunctional scaffold for anchoring numerous proteins and protein complexes. Through these interactions, pericentrin contributes to a diversity of fundamental cellular processes. Recent studies link pericentrin to a growing list of human disorders. Studies on pericentrin at the cellular, molecular, and, more recently, organismal level, provide a platform for generating models to elucidate the etiology of these disorders. Although the complexity of phenotypes associated with pericentrin-mediated disorders is somewhat daunting, insights into the cellular basis of disease are beginning to come into focus. In this review, we focus on human conditions associated with loss or elevation of pericentrin and propose cellular and molecular models that might explain them.
中心体是一种完整的组成部分，它作为一个多功能支架，用于固定许多蛋白质和蛋白质复合物。通过这些相互作用，中心体参与了多种基本的细胞过程。最近的研究将中心体与越来越多的人类疾病联系起来。在细胞、分子水平上的中心体研究，以及最近在生物体水平上的研究，为阐明这些疾病的病因提供了模型生成的平台。尽管与中心体介导的疾病相关的表型的复杂性有些令人望而却步，但对疾病的细胞基础的认识开始逐渐清晰。在这篇综述中，我们关注与中心体缺失或升高相关的人类疾病，并提出可能解释这些疾病的细胞和分子模型。
