RAG2 蛋白 C 末端的缺失导致小鼠淋巴样发育受损。
Deletion of the RAG2 C terminus leads to impaired lymphoid development in mice.
作者信息
Akamatsu Yoshiko, Monroe Robert, Dudley Darryll D, Elkin Sheryl K, Gartner Frank, Talukder Sadiqur R, Takahama Yousuke, Alt Frederick W, Bassing Craig H, Oettinger Marjorie A
机构信息
Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA.
出版信息
Proc Natl Acad Sci U S A. 2003 Feb 4;100(3):1209-14. doi: 10.1073/pnas.0237043100. Epub 2003 Jan 16.
The recombination-activating gene (RAG)1 and RAG2 proteins comprise the lymphocyte-specific components of the V(D)J recombinase and are required for the assembly of antigen-receptor variable-region genes. A mutant truncated RAG2 protein ("core" RAG2) lacking the C-terminal 144 amino acids, together with core RAG1, is able to mediate the basic biochemical steps required for V(D)J recombination in vitro and in transfected cell lines. Here we examine the effect of replacing the endogenous RAG2 locus in mice with core RAG2. These mice generate substantial numbers of B and T cells, demonstrating that the core RAG2 protein retains significant in vivo function. However, core RAG2 mice display a reduction in the total number of B and T cells, reflecting impaired lymphocyte development at the progenitor stage associated with reduced chromosomal V(D)J recombination. We discuss potential roles of the RAG2 C terminus in mediating rearrangement of endogenous antigen-receptor loci.
重组激活基因（RAG）1和RAG2蛋白构成V（D）J重组酶的淋巴细胞特异性成分，是抗原受体可变区基因组装所必需的。一种缺失C末端144个氨基酸的突变截短RAG2蛋白（“核心”RAG2）与核心RAG1一起，能够在体外和转染细胞系中介导V（D）J重组所需的基本生化步骤。在此，我们研究用核心RAG2替代小鼠内源性RAG2基因座的效果。这些小鼠产生大量的B细胞和T细胞，表明核心RAG2蛋白在体内保留了显著功能。然而，核心RAG2小鼠的B细胞和T细胞总数减少，反映出祖细胞阶段淋巴细胞发育受损，这与染色体V（D）J重组减少有关。我们讨论了RAG2 C末端在介导内源性抗原受体基因座重排中的潜在作用。
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