Phenotypic Features of Selective T Cell Deficiency Characterized by Absence of CD8+T Lymphocytes and Undetectable mRNA for ZAP-70 Kinase
| Autor: | Richard A. Franklin, Bruce Mazer, Reinhart Schwinzer, Ronald J. Harbeck, Anthony R. Hayward, Erwin W. Gelfand, Ralph T. Kubo |
|---|---|
| Rok vydání: | 1997 |
| Předmět: |
Male
T-Lymphocytes T cell Immunology Receptors Antigen T-Cell CD8-Positive T-Lymphocytes Biology Lymphocyte Activation Pathology and Forensic Medicine Interleukin 21 T-Lymphocyte Subsets medicine Humans Immunology and Allergy Cytotoxic T cell Lymphocyte Count RNA Messenger IL-2 receptor Antigen-presenting cell ZAP-70 Protein-Tyrosine Kinase Ionomycin ZAP70 Immunologic Deficiency Syndromes Infant CD28 T lymphocyte Protein-Tyrosine Kinases Flow Cytometry Molecular biology Phenotype medicine.anatomical_structure Calcium Signal Transduction |
| Zdroj: | Clinical Immunology and Immunopathology. 84:129-138 |
| ISSN: | 0090-1229 |
| Popis: | Selective T cell deficiency is a rare immune deficiency characterized by the absence of CD8 + T lymphocytes and depressed/absent T cell function. This syndrome has been associated with mutations in the gene for ZAP-70, a tyrosine kinase that has profound effects on signaling via the T cell receptor. In this paper we describe a patient with selective T cell deficiency and certain phenotypic features that are unique among the small number of patients described. The patient had virtually absent T cell function, hypogammaglobulinemia, and no response to vaccination. The T lymphocytes failed to respond to mitogenic stimuli, even in the presence of exogenous interleukin 2. Similar to other patients with this disorder, the T cells were capable of proliferating when stimulated by pharmacologic agents such as phorbol ester and ionomycin. While peripheral blood T cells had limited capability to increase cytosolic Ca 2+ levels in response to mitogenic stimulation, thymocytes responded to a large panel of antibodies and mitogens. This report broadens the spectrum of clinical presentations associated with selective T cell deficiency and, for the first time, compares the responses of both peripheral T cells and thymocytes. The data support the concept that the defect in signal transduction resulting from the absence of ZAP-70 is primarily manifested following export of T lymphocytes from the thymus and that selection of CD8-positive T cells is dependent on the presence of ZAP-70. |
| Databáze: | OpenAIRE |
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