[1]肖斌,李嘉颖,周梦思,等.B细胞连接蛋白的结构、功能及其在B细胞相关疾病发展中的作用[J].南方医科大学学报,2019,(02):253.[doi:10.12122/j.issn.1673-4254.2019.02.20]
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B细胞连接蛋白的结构、功能及其在B细胞相关疾病发展中的作用()
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《南方医科大学学报》[ISSN:1673-4254/CN:44-1627/R]

卷:
期数:
2019年02期
页码:
253
栏目:
出版日期:
2019-02-28

文章信息/Info

Title:
Structure and function of B-cell linker and its role in the development of B cell-related diseases
作者:
肖斌李嘉颖周梦思李晓晴黄晓燕杭建峰孙朝晖李林海
关键词:
B细胞连接蛋白B细胞受体信号通路分化B细胞相关疾病
Keywords:
B-cell linker B cell receptor signaling pathway differentiation B cell-related diseases
DOI:
10.12122/j.issn.1673-4254.2019.02.20
摘要:
B细胞连接蛋白,是B细胞受体通路的关键接头蛋白。B细胞连接蛋白通过自身的结构特性及蛋白互作网络参与调控 PLC-γ的活性和Ras通路激活,在B细胞受体信号转导中发挥重要作用,广泛参与调控B细胞的增殖、分化、凋亡及信号传导等过 程,并与过敏性疾病、多发性硬化症、染色体组非整倍体、无丙种球蛋白血症、B淋巴细胞白血病、淋巴瘤的发生发展密切相关。 本文对B细胞连接蛋白的结构、生物学功能及其在B细胞相关疾病中的作用进行综述:B细胞连接蛋白可与多个效应蛋白协作, 激活B细胞受体信号通路,从而影响着B细胞的发育成熟及功能调节;B细胞连接蛋白发生功能性突变,会破坏B细胞稳态并影 响B细胞发育成熟,从而导致B细胞相关疾病的发生。全面总结B细胞连接蛋白的生物学功能不仅有助于了解B细胞相关疾病 的发生机制,也有望将B细胞连接蛋白作为相关疾病的治疗靶标提供新思路和突破口。
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.
更新日期/Last Update: 1900-01-01