RAG and RAG defects

被引：47

作者：

Notarangelo, LD ^{[1
]}

Villa, A

Schwarz, K

机构：

[1] Univ Brescia, Spedali Civili, Dept Pediat, I-25122 Brescia, Italy

[2] CNR, Ist Tecnol Biomed Avanzate, I-20090 Segrate, MI, Italy

[3] Univ Ulm, Dept Transfus Med, D-89081 Ulm, Germany

来源：

CURRENT OPINION IN IMMUNOLOGY | 1999年 / 11卷 / 04期

关键词：

D O I：

10.1016/S0952-7915(99)80073-9

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

Products of the recombination-activating gene (RAG) play a crucial role in lymphoid cell development. During the past year, the functional properties of RAG protein domains have been better defined. Some mutations that alter the amino acid sequence of RAG1 or RAG2 have been shown to disturb B cell generation and to partially disturb T cell generation, resulting in Omenn syndrome in humans; moreover, peripheral re-expression of RAGs indicates their role in lymphoid cell homeostasis.

引用

页码：435 / 442

页数：8

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