鸟苷酸结合蛋白α亚基变异与腹膜假黏液瘤关系的研究进展

Research progress on the relationship between mutations in theα-subunit of guanine nucleotide-binding protein and pseudomyxoma peritonei

鸟苷酸结合蛋白(guanine nucleotide-binding protein,G蛋白)是细胞信号转导的关键调控因子。G蛋白结构和功能异常是诸多疾病的常见分子病理机制。α亚基是G蛋白结构和功能中心。腹膜假黏液瘤(pseudomyxoma peritonei,PMP)是一种以黏蛋白持续分泌为主要病理特征的恶性肿瘤综合征,Gα蛋白结构、功能变异导致黏液的持续分泌,是腹膜假黏液瘤的重要分子病理机制。本文就Gα蛋白基因表达、Gα蛋白变异的结构及功能特征、Gα蛋白变异与腹膜假黏液瘤相关性的研究进展进行综述,以期探索腹膜假黏液瘤发病的分子病理学机制。

Guanine nucleotide-binding proteins(G proteins)are crucial in the regulation of both intercellular and intracellular signal transduction.Structural mutations in and the dysfunction of G proteins are common molecular pathological mechanisms in many diseases.Theα-subunit is the structural and functional center of a G protein.Pseudomyxoma peritonei is a malignancy involving persistent mucin secretion.Structural and functional mutations in the Gαprotein result in the continuous secretion of mucus,an important molecular pathological mechanism of pseudomyxoma peritonei.This review summarizes the expression pattern of the Gαgene,the structural and functional changes in the Gαprotein after mutation,and the relationship between Gαmutations and pseudomyxoma peritonei,in order to explore the molecular pathological mechanism of pseudomyxoma peritonei.