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[B细胞连接蛋白的结构与功能及其在B细胞相关疾病发生发展中的作用]

[Structure and function of B-cell linker and its role in the development of B cell-related diseases].

作者信息

Xiao Bin, Li Jiaying, Zhou Mengsi, Li Xiaoqing, Huang Xiaoyan, Hang Jianfeng, Sun Zhaohui, Li Linhai

机构信息

Department of Laboratory Medicine, General Hospital of Southern Theatre Command of PLA, Guangzhou 510010, China.

Department of Clinical Laboratory, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.

出版信息

Nan Fang Yi Ke Da Xue Xue Bao. 2019 Feb 28;39(2):253-256. doi: 10.12122/j.issn.1673-4254.2019.02.20.

Abstract

B cell linker (BLNK) is a key linker protein of B cell receptor (BCR) signaling pathway. BLNK participates in the regulation of PLC-γactivity and the activation of Ras pathway through its typical structure and interaction network with other proteins, and is thus widely involved in the regulation of B cell proliferation, differentiation, apoptosis and signal transduction. Furthermore, it is closely related to anaphylactic diseases, multiple sclerosis, chromosomal aneuploidy, aneuglobulinemia, B lymphocytic leukemia and lymphoma. Herein we review the structure and biological function of BLNK and its role in B cell-related diseases. BLNK can cooperate with a series of effective proteins to activate BCR signaling pathway, thereby regulating the development, maturation and function of B cells. The functional mutation of BLNK can destroy the homeostasis of B cells and affect the development and maturation of B cells, which leads to the occurrence of B cell related diseases. A comprehensive understanding of the biological functions of BLNK not only provides insights into the pathogenesis of B cell-related diseases, but also inspires new ideas and helps to find breakthroughs for the treatment of these diseases with BLNK as the therapeutic target.

摘要

B细胞连接蛋白(BLNK)是B细胞受体(BCR)信号通路中的关键连接蛋白。BLNK通过其典型结构以及与其他蛋白质的相互作用网络,参与对磷脂酶C-γ(PLC-γ)活性的调节和Ras通路的激活,因而广泛参与B细胞增殖、分化、凋亡及信号转导的调控。此外,它与过敏性疾病、多发性硬化症、染色体非整倍性、无球蛋白血症、B淋巴细胞白血病及淋巴瘤密切相关。在此,我们综述BLNK的结构和生物学功能及其在B细胞相关疾病中的作用。BLNK可与一系列效应蛋白协同作用以激活BCR信号通路,从而调节B细胞的发育、成熟及功能。BLNK的功能突变可破坏B细胞的稳态并影响B细胞的发育和成熟,进而导致B细胞相关疾病的发生。全面了解BLNK的生物学功能不仅有助于深入认识B细胞相关疾病的发病机制,还能启发新思路并有助于以BLNK作为治疗靶点为这些疾病的治疗找到突破口。

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