Browse by author
Lookup NU author(s): Dr Andrew McLean-Tooke, Dr Gavin Spickett, Professor Andrew GenneryORCiD
Full text for this publication is not currently held within this repository. Alternative links are provided below where available.
In 22q11.2 deletion patients, the normal decrease in T lymphocyte counts after 1-2 years is blunted such that relatively T lymphocyte numbers increase over early childhood, probably via post-thymic expansion of peripheral lymphocytes. This may leave less T lymphocyte receptor (TCR) diversity than when derived from naive thymic emigrants. We analysed TCR V beta repertoire on 27 22q11.2 chromosome deletion patients. No patient had infection at sampling. CD3+CD4+ recent thymic emigrants (RTEs) were identified by CD45RA and CD31 expression. TCR V beta repertoire was determined using four-colour flow cytometry. Patients and controls showed significant TCR V beta family usage differences between CD3+CD4+ and CD3+CD4- T lymphocyte subpopulations. V beta family abnormalities (+/- 3 SD of controls) were identified in 18/27 (67%) patients and 12/47 (25%) controls. In patients, the magnitude of expansions was increased, with some V beta families representing 37% of the cells present in the subpopulations. There was a significant increase in frequency of abnormalities in CD3+CD4+ (P < 0.001) and CD3+CD4- T lymphocytes (P < 0.05) in patients. A total of 11/16 patients had an abnormal CD4+CD25Bright TCR V beta repertoire. There was no difference in expansions/contractions between CD4+CD25Bright and CD4+ T lymphocyte repertoires (P = 0.575) for individual patients but significant differences in expansions/contractions between CD4+CD25Bright and CD8+ T lymphocytes repertoires (P = 0.011). There was bias in V beta usage between CD3+CD4+ and CD3+CD4- T lymphocyte subsets. A total of 67% patients had TCR V beta repertoire abnormalities, with a trend towards increased repertoire abnormalities with fewer RTEs, suggesting thymic output plays an important role in TCR repertoire diversity. There was no correlation between skewed repertoire and symptoms of infection or autoimmunity.
Author(s): McLean-Tooke A, Barge D, Spickett GP, Gennery AR
Publication type: Article
Publication status: Published
Journal: Scandinavian Journal of Immunology
Year: 2011
Volume: 73
Issue: 6
Pages: 577-585
Print publication date: 27/04/2011
ISSN (print): 0300-9475
ISSN (electronic): 1365-3083
Publisher: Wiley-Blackwell
URL: http://dx.doi.org/10.1111/j.1365-3083.2011.02527.x
DOI: 10.1111/j.1365-3083.2011.02527.x
Altmetrics provided by Altmetric
