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B 细胞发育中的遗传缺陷及其临床后果。

Genetic defects in B-cell development and their clinical consequences.

出版信息

J Investig Allergol Clin Immunol. 2014;24(1):6-22; quiz 2 p following 22.

PMID:24765876
Abstract

Expression of selected genes in hematopoietic stem cells has been identified as a regulator of differentiation of B cells in the liver and bone marrow. Moreover, naïve B cells expressing surface immunoglobulin need other types of genes for antigen-dependent development in secondary lymphoid organs. Many advanced molecular mechanisms underlying primary antibody deficiencies in humans have been described. We provide an overview of the mutations in genes known to be involved in B-cell development and their clinical consequences.

摘要

造血干细胞中特定基因的表达被鉴定为调节肝和骨髓中 B 细胞分化的因素。此外,表达表面免疫球蛋白的幼稚 B 细胞需要其他类型的基因,以便在次级淋巴器官中进行抗原依赖性发育。已经描述了许多人类原发性抗体缺陷背后的先进分子机制。我们概述了已知参与 B 细胞发育的基因中的突变及其临床后果。

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