Pericentrin in cellular function and disease.

Pericentrin in cellular function and disease.
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DOI:
10.1083/jcb.200908114
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发表时间:
2010-01-25
期刊:
The Journal of cell biology
影响因子:
--
通讯作者:
Doxsey SJ
Doxsey SJ
中科院分区:
其他
文献类型:
--
作者:
Delaval B;Doxsey SJ

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Pericentrin是中心体的一个组成部分,作为锚定许多蛋白质和蛋白质复合物的多功能支架。通过这些相互作用,pericentrin有助于基本细胞过程的多样性。最近的研究将pericentrin与越来越多的人类疾病联系起来。在细胞,分子,以及最近,生物体水平上的pericentrin的研究,提供了一个平台,产生模型,以阐明这些疾病的病因。虽然与围中心蛋白介导的疾病相关的表型的复杂性有些令人生畏,但对疾病的细胞基础的见解开始成为焦点。在这篇综述中,我们专注于与pericentrin的损失或升高相关的人类条件,并提出可能解释它们的细胞和分子模型。
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.
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