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Down syndrome (DS) peripheral blood contains phenotypically mature CD3+TCR alpha, beta+ cells but abnormal proportions of TCR alpha, beta+, TCR gamma, delta+, and CD4+ CD45RA+ cells: evidence for an inefficient release of mature T cells by the DS thymus.
Clin Immunol Immunopathol. 1992 Feb; 62(2):245-51.CI

Abstract

Down syndrome (DS) thymocytes have a markedly diminished proportion of cells expressing high levels of the alpha, beta T cell receptor (TCR alpha, beta) and the associated CD3 molecule. Thus, we examined the surface expression of TCR alpha, beta and CD3 as well as TCR gamma, delta, CD4, CD8, CD16, and CD45RA on peripheral blood lymphocytes (PBL) from 13 noninstitutionalized subjects with DS and 13 closely age-matched sibling controls using immunofluorescence and flow cytometry. DS PBL expressed high surface levels of TCR alpha, beta and CD3, but, as compared to controls, they had a lower proportion of cells expressing TCR alpha, beta (61% vs. 68%, respectively; P less than or equal to 0.05). Moreover, the absolute number of TCR alpha, beta+ cells was considerably lower for DS subjects than for controls (1634 +/- 229 vs. 2763 +/- 530, respectively; P less than or equal to 0.05). DS subjects had a markedly higher proportion of cells expressing TCR gamma, delta than did the controls (12% vs. 7%, respectively; P less than or equal to 0.02). In addition, DS subjects had a lower proportion of CD4+CD45RA+ cells than controls (22% vs. 35%, respectively; P less than or equal to 0.02), representing naive T cells which have recently emigrated from the thymus. The imbalance in the proportions of T cell subpopulations we have observed in DS PBL may contribute to the increased susceptibility to infection associated with DS and may represent a diminished efficiency in the production of newly differentiated T cells by the DS thymus.

Authors+Show Affiliations

Department of Pediatrics, University of California, San Francisco 94143.No affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

1530912

Citation

Murphy, M, and L B. Epstein. "Down Syndrome (DS) Peripheral Blood Contains Phenotypically Mature CD3+TCR Alpha, Beta+ Cells but Abnormal Proportions of TCR Alpha, Beta+, TCR Gamma, Delta+, and CD4+ CD45RA+ Cells: Evidence for an Inefficient Release of Mature T Cells By the DS Thymus." Clinical Immunology and Immunopathology, vol. 62, no. 2, 1992, pp. 245-51.
Murphy M, Epstein LB. Down syndrome (DS) peripheral blood contains phenotypically mature CD3+TCR alpha, beta+ cells but abnormal proportions of TCR alpha, beta+, TCR gamma, delta+, and CD4+ CD45RA+ cells: evidence for an inefficient release of mature T cells by the DS thymus. Clin Immunol Immunopathol. 1992;62(2):245-51.
Murphy, M., & Epstein, L. B. (1992). Down syndrome (DS) peripheral blood contains phenotypically mature CD3+TCR alpha, beta+ cells but abnormal proportions of TCR alpha, beta+, TCR gamma, delta+, and CD4+ CD45RA+ cells: evidence for an inefficient release of mature T cells by the DS thymus. Clinical Immunology and Immunopathology, 62(2), 245-51.
Murphy M, Epstein LB. Down Syndrome (DS) Peripheral Blood Contains Phenotypically Mature CD3+TCR Alpha, Beta+ Cells but Abnormal Proportions of TCR Alpha, Beta+, TCR Gamma, Delta+, and CD4+ CD45RA+ Cells: Evidence for an Inefficient Release of Mature T Cells By the DS Thymus. Clin Immunol Immunopathol. 1992;62(2):245-51. PubMed PMID: 1530912.
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TY - JOUR T1 - Down syndrome (DS) peripheral blood contains phenotypically mature CD3+TCR alpha, beta+ cells but abnormal proportions of TCR alpha, beta+, TCR gamma, delta+, and CD4+ CD45RA+ cells: evidence for an inefficient release of mature T cells by the DS thymus. AU - Murphy,M, AU - Epstein,L B, PY - 1992/2/1/pubmed PY - 1992/2/1/medline PY - 1992/2/1/entrez SP - 245 EP - 51 JF - Clinical immunology and immunopathology JO - Clin Immunol Immunopathol VL - 62 IS - 2 N2 - Down syndrome (DS) thymocytes have a markedly diminished proportion of cells expressing high levels of the alpha, beta T cell receptor (TCR alpha, beta) and the associated CD3 molecule. Thus, we examined the surface expression of TCR alpha, beta and CD3 as well as TCR gamma, delta, CD4, CD8, CD16, and CD45RA on peripheral blood lymphocytes (PBL) from 13 noninstitutionalized subjects with DS and 13 closely age-matched sibling controls using immunofluorescence and flow cytometry. DS PBL expressed high surface levels of TCR alpha, beta and CD3, but, as compared to controls, they had a lower proportion of cells expressing TCR alpha, beta (61% vs. 68%, respectively; P less than or equal to 0.05). Moreover, the absolute number of TCR alpha, beta+ cells was considerably lower for DS subjects than for controls (1634 +/- 229 vs. 2763 +/- 530, respectively; P less than or equal to 0.05). DS subjects had a markedly higher proportion of cells expressing TCR gamma, delta than did the controls (12% vs. 7%, respectively; P less than or equal to 0.02). In addition, DS subjects had a lower proportion of CD4+CD45RA+ cells than controls (22% vs. 35%, respectively; P less than or equal to 0.02), representing naive T cells which have recently emigrated from the thymus. The imbalance in the proportions of T cell subpopulations we have observed in DS PBL may contribute to the increased susceptibility to infection associated with DS and may represent a diminished efficiency in the production of newly differentiated T cells by the DS thymus. SN - 0090-1229 UR - https://www.unboundmedicine.com/medline/citation/1530912/Down_syndrome__DS__peripheral_blood_contains_phenotypically_mature_CD3+TCR_alpha_beta+_cells_but_abnormal_proportions_of_TCR_alpha_beta+_TCR_gamma_delta+_and_CD4+_CD45RA+_cells:_evidence_for_an_inefficient_release_of_mature_T_cells_by_the_DS_thymus_ L2 - http://www.diseaseinfosearch.org/result/2327 DB - PRIME DP - Unbound Medicine ER -