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【综述】V(D)J基因重排分子缺陷与先天性免疫缺陷病的关系


  09-06- 12  


 

摘要先天性免疫是人体免疫系统抗感染的第一道防线。在脊椎动物,适应性免疫在防范病原起重要作用,依赖于产生各种类型的可溶性膜结合抗原受体,而这些抗原受体表达在T、B淋巴细胞表面。免疫球蛋白(Ig)和T细胞受体(TCR)的可变区域是一些基因片段编码。免疫多样性的实现是依赖于Ig和TCR可变区的V(D)J基因重排。本文就参与V(D)J基因重排的RAG1/2、Artemis、DNA连接酶IV和CemunnosXLF等分子的结构和功能以及这些分子缺陷与先天性免疫缺陷病的关系作一总结。
关键词V(D)J基因重排先天性免疫缺陷病分子缺陷
Relationship between molecular deficiency of V(D)J gene rearrangement and genetic immunodeficiency diseases
MA JunfangZHOU DinganHE Lin
ABSTRACTInnate immunity is primary defense mechanism of human immune system. In vertebrates, adaptive immunity plays an important role in precaution against pathogeny, which is dependant on the generation of various type of dissolvable conjugated antigen receptor of cell membrane, expressing on the cell surface of T, B lymphocytes. The variable regions of immunoglobulin (Ig) and T cell receptor (TCR) are encoded by some gene segments and V(D)J gene rearrangement of variable regions of Ig and TCR are required for the accomplishment of immune diversity. In this article, we intend to conclude the molecule constructure and function of RAG1/2, Artemis, DNAlingaseIV and CemunnosXLF etc which participate in V(D)J gene rearrangement and further illustrate the relationship between defencicy of these molecule above and genetic immunodeficiency diseases.
KEY WORDSV(D)J gene rearrangementGenetic immunodeficiency diseaseMolecular defeciency

作者单位1. 四川省乐山市红十字会医院检验科
2. 复旦大学生物医学研究院发育生物学与出生缺陷研究所


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