| Autor: |
Somberg RL; Department of Clinical Studies, School of Veterinary Medicine, University of Pennsylvania, Philadelphia 19104., Robinson JP, Felsburg PJ |
| Jazyk: |
angličtina |
| Zdroj: |
Journal of immunology (Baltimore, Md. : 1950) [J Immunol] 1994 Nov 01; Vol. 153 (9), pp. 4006-15. |
| Abstrakt: |
Canine X-linked severe combined immunodeficiency disease (XSCID) is characterized by a failure to thrive, thymic dysplasia, and a lack of a T lymphocyte mitogenic response. As in human XSCID, affected dogs in our colony have a mutation in the IL-2R-gamma gene. This mutation dramatically altered T lymphocyte development, because XSCID thymi were severely reduced in size and cellularity, contained an increased proportion of immature CD4-CD8- thymocytes, a decreased proportion of intermediate CD4+CD8+ thymocytes, and a normal proportion of CD4+CD8- and CD4-CD8+ thymocytes. XSCID thymi were also deficient in the percentage of CD3-L+ thymocytes. Interestingly, several XSCID dogs had normal percentages of CD3-L+ PBL. Although the mutation did not interfere with IL-2 production, PHA-activated XSCID PBL demonstrated severely diminished IL-2 binding and were nonresponsive to IL-2. These results indicate that the lack of a functional IL-2R-gamma chain in dogs with XSCID primarily affects developing CD4-CD8- thymocytes as they acquire the cell surface Ag CD3-L and interferes with the ability of peripheral T lymphocytes to bind and respond to IL-2. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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