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The scid mutation in mice causes a general defect in DNA repair

Nature volume 347, pages 479–482 (1990)Cite this article

Abstract

MICE homozygous for the scid mutation on chromosome 16 have a severe combined immune deficiency1,2 as a result of their inability to correctly rearrange their immunoglobulin and T-cell receptor genes3,4. In scid mice, when precursors for B and T lymphocytes reach the stage of development requiring expression of these surface receptors, a defective recombinase system aberrantly cuts and rejoins the receptor gene segments greatly reducing the efficiency of producing functional receptors. As a result, most scid mice have no detectable B or T lymphocytes. We have demonstrated that the scid defect is not specific to lymphocyte development. Myeloid cells and fibroblasts from scid mice show a marked increase in sensitivity to ionizing radiation, indicating that the scid mutation leads to an inability to repair DNA damage induced by ionizing radiation as well as interfering with rearrangement of the immunoglobulin and T-cell receptor genes.

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Author notes

  1. R. A. Phillips: To whom correspondence should be addressed.

Authors and Affiliations

  1. Division of Immunology and Cancer Research, Research Institute, Hospital for Sick Children and Departments of Medical Genetics and Immunology, University of Toronto, Toronto, Canada, M5G 1X8

    G. M. Fulop & R. A. Phillips

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  1. G. M. Fulop
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  2. R. A. Phillips
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Fulop, G., Phillips, R. The scid mutation in mice causes a general defect in DNA repair. Nature 347, 479–482 (1990). https://doi.org/10.1038/347479a0

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