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Postnatal development of T cells in dogs with X-linked severe combined immunodeficiency.

R L Somberg, A Tipold, B J Hartnett, P F Moore, P S Henthorn and P J Felsburg
J Immunol February 15, 1996, 156 (4) 1431-1435;
R L Somberg
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A Tipold
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B J Hartnett
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P F Moore
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P S Henthorn
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P J Felsburg
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Abstract

X-linked severe combined immunodeficiency disease (XSCID) in both humans and dogs results from mutations in the common gamma-chain, gamma c, which is a common component of the receptors for IL-2, IL-4, IL-7, IL-9, and IL-15. Although human and canine XSCID share similar features, such as a failure to thrive, hypogammaglobulinemia, an absent T cell mitogenic response, and thymic dysplasia, near normal percentages of T cells are observed in some affected dogs, whereas XSCID boys have few, if any, circulating T cells. In this study, PBL were analyzed by flow cytometry beginning shortly after birth until 9 wk of age. XSCID dogs < 3 wk of age had an elevated number of B cells and were nearly devoid of T cells, phenotypically resembling most human XSCID patients. At 5 wk of age, however, T cells appeared in approximately one-half of the XSCID dogs, although the absolute number of T cells was one-third of normal in these dogs. While the percentage of CD45RA+ T cells in normal dogs gradually decreased with age from > 90% in neonates to < 40% by 3 to 5 yr of age, in XSCID dogs a rapid decline in the percentage of CD45RA+ T cells was observed, resulting in < 10% CD45RA+ T cells by 7 to 9 wk of age. Maternal engraftment was not detected in any of the XSCID dogs by using a sensitive PCR assay. The appearance of nonmaternally derived T cells in XSCID dogs that undergo a rapid switch from CD45RA+ to CD45RA- suggests that limited thymic emigration and peripheral expansion of T cells can occur in the absence of a functional gamma c.

  • Copyright © 1996 by American Association of Immunologists

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The Journal of Immunology
Vol. 156, Issue 4
15 Feb 1996
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Postnatal development of T cells in dogs with X-linked severe combined immunodeficiency.
R L Somberg, A Tipold, B J Hartnett, P F Moore, P S Henthorn, P J Felsburg
The Journal of Immunology February 15, 1996, 156 (4) 1431-1435;

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Postnatal development of T cells in dogs with X-linked severe combined immunodeficiency.
R L Somberg, A Tipold, B J Hartnett, P F Moore, P S Henthorn, P J Felsburg
The Journal of Immunology February 15, 1996, 156 (4) 1431-1435;
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Print ISSN 0022-1767        Online ISSN 1550-6606