Clinical consequences of defects in B-cell development.
Clinical consequences of defects in B-cell development.
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DOI：
10.1016/j.jaci.2010.02.018
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发表时间：
2010-04
影响因子：
14.2
通讯作者：
Schroeder, Harry W., Jr.
中科院分区：
文献类型：
--
作者：
Vale, Andre M.;Schroeder, Harry W., Jr.
体液免疫的缺失通常反映有效B细胞发育的普遍或选择性失败。发育过程可以通过分析细胞表面标志物如IgM、IgD、CD10、CD19、CD20、CD21和CD38来跟踪。B细胞发育的早期阶段致力于产生免疫球蛋白和测试B细胞抗原受体信号传导。失败导致出生时血液中缺乏B细胞和免疫球蛋白。当发育中的B细胞开始表达表面B细胞受体时，它们变得受到阴性和阳性选择压力的影响，并且越来越依赖于存活信号。即使在B细胞数量正常的情况下，信号传导缺陷也可导致选择性或全身性低丙种球蛋白血症。在次级淋巴器官中，一些B细胞进入脾边缘区，在那里预活化的细胞准备好对T非依赖性抗原（如包被某些微生物的多糖）快速应答。其他细胞进入滤泡，并在同源滤泡T细胞的帮助下，在与抗原相互作用后分裂以帮助形成生发中心。在生发中心，B细胞可以经历类别转换和体细胞超变的过程。不能正确接收T细胞信号可能导致高IgM综合征。离开生发中心的B细胞可以发育成记忆B细胞、短寿命浆细胞或长寿命浆细胞。后者最终迁移回骨髓，在那里它们可以继续产生保护性的抗原特异性抗体数十年。
Abnormalities in humoral immunity typically reflect a generalized or selective failure of effective B cell development. The developmental processes can be followed through analysis of cell surface markers such as IgM, IgD, CD10, CD19, CD20, CD21, and CD38. Early phases of B cell development are devoted to the creation of immunoglobulin and testing B cell antigen receptor signaling. Failure leads to the absence of B cells and immunoglobulin in the blood from birth. As the developing B cells begin to express a surface B cell receptor, they become subject to negative and positive selection pressures and increasingly depend on survival signals. Defective signalling can lead to selective or generalized hypogammaglobulinemia even in the presence of normal numbers of B cells. In the secondary lymphoid organs, some B cells enter the splenic marginal zone where pre-activated cells lie ready to rapidly respond to T-independent antigens, such as the polysaccharides that coat some microorganisms. Other cells enter the follicle and, with the aid of cognate follicular T cells, divide to help form a germinal center after their interaction with antigen. In the germinal center, B cells can undergo the processes of class switching and somatic hypermutation. Failure to properly receive T cell signals can lead to Hyper IgM syndrome. B cells that leave the germinal center can develop into memory B cells, short lived plasma cells, or long lived plasma cells. The latter ultimately migrate back to the bone marrow where they can continue to produce protective antigen-specific antibodies for decades.
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DOI：
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影响因子：
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影响因子：
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