摘要SHARPIN是新近发现的一种线性泛素化链组装复合物的组成元件,已有大量研究证实SHARPIN调控多种生物信号通路,参与多种生理过程,近年来已成为生物医学的研究热点:参与调控IL-1、TNF-α等炎症因子刺激介导的NFκB及细胞凋亡信号通路,抑制β1-整合素的激活；SHARPIN功能丧失导致小鼠多器官嗜酸性粒细胞浸润为主的炎症损害、免疫系统紊乱等；SHARPIN在多种内脏肿瘤组织中表达升高,并在体外实验中显示出癌基因特性.该文就SHARPIN基因的结构、生物功能与网络、相关疾病动物模型等作一简要综述.

abstractsSHARPIN is a newly identified component of the linear ubiquitin chain assembly complex (LUBAC) demonstrating critical roles in multiple cellular signaling and physiological events.Recent biomedical studies have revealed that SHARPIN plays important roles in IL-1 and/or TNF-αinduced activation of NFκB pathway,apoptosis and β1-integrin signaling.Loss of function of SHARPIN leads to multi-organ defects characterized by eosinophilic inflammation and dysregulation of immune system.Expression of SHARPIN was upregnlated also in tumors of different tissue origin,which implicated oncogene features.Herein we briefly introduced the gene structure,biological function and network,and animal models of disease related to SHARPIN.