Repertoire of Vα and Vβ regions of T cell antigen receptors on CD4+ and CD8+ peripheral blood T cells in a novel X-linked combined immunodeficiency disease
| Autor: | Armond S. Goldman, Frank C. Schmalstieg, Edward G. Brooks, Helen E. Rudloff, Kimberly H. Palkowetz |
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| Rok vydání: | 1992 |
| Předmět: |
CD4-Positive T-Lymphocytes
Male X Chromosome CD8 Antigens Receptors Antigen T-Cell alpha-beta T cell Immunology T-cell receptor Immunologic Deficiency Syndromes Biology Flow Cytometry medicine.disease Natural killer T cell Interleukin 21 medicine.anatomical_structure T-Lymphocyte Subsets medicine Humans Immunology and Allergy Cytotoxic T cell X-linked severe combined immunodeficiency Immunodeficiency CD8 |
| Zdroj: | European Journal of Immunology. 22:1103-1106 |
| ISSN: | 1521-4141 0014-2980 |
| DOI: | 10.1002/eji.1830220435 |
| Popis: | The repertoire of V regions of alpha/beta+ T cell receptor (TcR) on CD4+ and CD8+ T cells from the peripheral blood of six patients with a novel X-linked combined immunodeficiency was investigated by flow cytometry. The relative frequencies of V region subfamilies V alpha 2a on CD4+ T lymphocytes and V beta 5b and V beta 12a on CD8+ T lymphocytes from the peripheral blood from the affected males were decreased significantly. Also, the relative frequencies of certain other V region subfamilies on CD4+ or CD8+ T cells from the peripheral blood of some patients were either considerably below or above the ranges found in normal subjects. Although there may be other explanations including an extrathymic event, we suggest that the abnormalities in the TcR repertoire of peripheral blood T cells are consistent with a dysregulation of the intrathymic maturation/selection of T cells that leads to deficiencies in T cell populations in the peripheral blood of males with this disease. |
| Databáze: | OpenAIRE |
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