A severe combined immunodeficiency mutation in the mouse (original) (raw)
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- Published: 10 February 1983
Nature volume 301, pages 527–530 (1983)Cite this article
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Abstract
The most debilitating human lymphoid deficiency disease, known as severe combined immunodeficiency (SCID), impairs the differentiation of both T and B lymphocytes1–7. Affected infants are highly susceptible to recurring infections of viruses, fungi and bacteria and invariably die within 2yr of birth. Inheritance of this congenital syndrome may show X-linked8,9 or autosomal recessive control1,2,9. To date autosomal recessive inheritance of SCID has been observed in Arabian foals10 which represent the only known animal model of this disease syndrome but here we report an autosomal recessive mutation in mice that severely impairs lymphopoiesis. Mice homozygous for this mutation have few if any lymphocytes; consequently they are hypogammaglobulinaemic and deficient for immune functions mediated by T and B lymphocytes. These mice, therefore, represent a new model for investigating how lymphoid differentiation may be impaired in the disease state and regulated in the normal state.
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Authors and Affiliations
- Institute for Cancer Research, Fox Chase Cancer Center, Philadelphia, Pennsylvania, 19111, USA
Gayle C. Bosma, R. Philip Custer & Melvin J. Bosma
Authors
- Gayle C. Bosma
You can also search for this author inPubMed Google Scholar - R. Philip Custer
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Bosma, G., Custer, R. & Bosma, M. A severe combined immunodeficiency mutation in the mouse.Nature 301, 527–530 (1983). https://doi.org/10.1038/301527a0
- Received: 21 September 1982
- Accepted: 24 November 1982
- Issue Date: 10 February 1983
- DOI: https://doi.org/10.1038/301527a0