RAG-1-deficient mice have no mature B and T lymphocytes

doi:10.1016/0092-8674(92)90030-G

Cell

Volume 68, Issue 5, 6 March 1992, Pages 869-877

https://doi.org/10.1016/0092-8674(92)90030-G Get rights and content

Abstract

The V(D)J recombination activation gene RAG-1 was isolated on the basis of its ability to activate V(D)J recombination on an artificial substrate in fibroblasts. This property and the expression pattern in tissues and cell lines indicate that RAG-1 either activates or catalyzes the V(D)J recombination reaction of immunoglobulin and T cell receptor genes. We here describe the introduction of a mutation in RAG-1 into the germline of mice via gene targeting in embryonic stem cells. RAG-1-deficient mice have small lymphoid organs that do not contain mature B and T lymphocytes. The arrest of B and T cell differentiation occurs at an early stage and correlates with the inability to perform V(D)J recombination. The immune system of the RAG-1 mutant mice can be described as that of nonleaky scid mice. Although RAG-1 expression has been reported in the central nervous system of the mouse, no obvious neuroanatomical or behavioral abnormalities have been found in the RAG-1-deficient mice.

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Cell

ArticleRAG-1-deficient mice have no mature B and T lymphocytes

Abstract

Gene

Current Biology

Cell

Cell

Cell

Cell. Immunol.

Cell

Curr. Op. Immunol.

Cell

Cell

Cell

Cell

Cell

Regulation of genome rearrangement events during lymphocyte development

Immunol. Rev.

Scid mutation in mice confers hypersensitivity to ionizing radiation and a deficiency in DNA double-strand break repair

A simple technique for generating probes for RNA in situ hybridization: an adjunct to genome mapping exemplified by the RAG-1RAG-2 gene cluster

The scid mouse mutant: definition, characterization and potential uses

Annu. Rev. Immunol.

A severe combined immunodeficiency mutation in the mouse

Nature

Evidence of functional lymphocytes in some (leaky) scid mice

J. Exp. Med.

Production and analysis of chimeric mice

Altering the genome by homologous recombination

Science

T-lymphocyte development in scid mice is arrested shortly after the initiation of T-cell receptor δ gene recombination

Genes Dev.

Detection and characterization of functional T cells in mice with severe combined immune deficiency

Eur. J. Immunol.

Occurrence of mature B (IgM+,B220+) and T (CD3+) lymphocytes in scid mice

J. Immunol.

T-cell receptor δ gene rearrangements in early thymocytes

Nature

Article
RAG-1-deficient mice have no mature B and T lymphocytes

A simple technique for generating probes for RNA in situ hybridization: an adjunct to genome mapping exemplified by the $RAG-1 RAG-2$ gene cluster

Occurrence of mature B (IgM⁺,B220⁺) and T (CD3⁺) lymphocytes in scid mice