Department of Medicine, Washington University, St Louis, MO, USA.
Organogenesis. 2013 Apr-Jun;9(2):61-9. doi: 10.4161/org.25144. Epub 2013 Apr 1.
The centrosome is the main microtubule-organizing center in animal cells, and helps to influence the morphology of the microtubule cytoskeleton in interphase and mitosis. The centrosome also templates the assembly of the primary cilium, and together they serve as a nexus of cell signaling that provide cells with diverse organization, motility, and sensory functions. The majority of cells in the human body contain a solitary centrosome and cilium, and cells have evolved regulatory mechanisms to precisely control the numbers of these essential organelles. Defects in the structure and function of cilia lead to a variety of complex disease phenotypes termed ciliopathies, while dysregulation of centrosome number has long been proposed to induce genome instability and tumor formation. Here, we review recent findings that link centrosome amplification to changes in cilium number and signaling capacity, and discuss how supernumerary centrosomes may be an important aspect of a set of cilia-related disease phenotypes.
中心体是动物细胞中主要的微管组织中心，有助于影响有丝分裂和间期的微管细胞骨架的形态。中心体还模板初级纤毛的组装，它们共同作为细胞信号转导的枢纽，为细胞提供多种组织、运动和感觉功能。人体中的大多数细胞含有一个单独的中心体和纤毛，细胞已经进化出调节机制来精确控制这些必需细胞器的数量。纤毛的结构和功能缺陷导致多种复杂的疾病表型，称为纤毛病，而中心体数量的失调很早就被提出会诱导基因组不稳定和肿瘤形成。在这里，我们回顾了最近的发现，这些发现将中心体扩增与纤毛数量和信号转导能力的变化联系起来，并讨论了多余的中心体如何可能是一组与纤毛相关的疾病表型的一个重要方面。
