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中心粒周蛋白(pericentrin,PCNT)是一种高度保守的广泛存在于动物和人体组织细胞中的蛋白,是中心粒周围物质(pericentriolar material,PCM)的组成成分之一。PCNT在细胞的周期进程和信号传导中发挥关键作用,包括参与调控中心体的结构和功能,参与有丝分裂期纺锤体形成和微管成核等,其异常表达与Ⅱ型骨发育不良性原发性侏儒症、糖代谢异常、恶性肿瘤、精神类、唐氏综合征、纤毛类等多种疾病的发生有关。近期有研究表明,其对胰岛B细胞的胰岛素分泌也有一定的影响。本文对PCNT的结构与功能及其相关疾病的发病机制的研究进展作一综述。
Pericentrin (PCNT) is a highly conserved protein widely found in animal and human tissue cells. It is one of the components of the pericentriolar material (PCM). PCNT plays a key role in the cell cycle progression and signal transduction, including the regulation of the structure and function of the central body, the formation of spindle in mitosis and the nucleation of microtubules. The abnormal expression of PCNT is associated with the development of primary dysplasia Dwarfism, abnormal glucose metabolism, malignant tumors, mental disorders, Down’s syndrome, cilia and other diseases. Recent studies have shown that it also has a certain impact on the insulin secretion of pancreatic B cells. This review summarizes the research progress of the structure and function of PCNT and the pathogenesis of its related diseases.