{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T04:13:35Z","timestamp":1790396015538,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2011,4,27]],"date-time":"2011-04-27T00:00:00Z","timestamp":1303862400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Scand J Immunol"],"published-print":{"date-parts":[[2011,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In 22q11.2 deletion patients, the normal decrease in T lymphocyte counts after 1\u20132\u2003years is blunted such that relatively T lymphocyte numbers increase over early childhood, probably via post\u2010thymic expansion of peripheral lymphocytes. This may leave less T lymphocyte receptor (TCR) diversity than when derived from naive thymic emigrants. We analysed TCR V\u03b2 repertoire on 27 22q11.2 chromosome deletion patients. No patient had infection at sampling. CD3<jats:sup>+<\/jats:sup>CD4<jats:sup>+<\/jats:sup> recent thymic emigrants (RTEs) were identified by CD45RA and CD31 expression. TCR V\u03b2 repertoire was determined using four\u2010colour flow cytometry. Patients and controls showed significant TCR V\u03b2 family usage differences between CD3<jats:sup>+<\/jats:sup>CD4<jats:sup>+<\/jats:sup> and CD3<jats:sup>+<\/jats:sup>CD4<jats:sup>\u2212<\/jats:sup> T lymphocyte subpopulations. V\u03b2 family abnormalities (\u00b13\u2003SD 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\u03b2 families representing 37% of the cells present in the subpopulations. There was a significant increase in frequency of abnormalities in CD3<jats:sup>+<\/jats:sup>CD4<jats:sup>+<\/jats:sup> (<jats:italic>P<\/jats:italic>\u2003&lt;\u20030.001) and CD3<jats:sup>+<\/jats:sup>CD4<jats:sup>\u2212<\/jats:sup> T lymphocytes (<jats:italic>P<\/jats:italic>\u2003&lt;\u20030.05) in patients. A total of 11\/16 patients had an abnormal CD4<jats:sup>+<\/jats:sup>CD25<jats:sup>Bright<\/jats:sup> TCR V\u03b2 repertoire. There was no difference in expansions\/contractions between CD4<jats:sup>+<\/jats:sup>CD25<jats:sup>Bright<\/jats:sup> and CD4<jats:sup>+<\/jats:sup> T lymphocyte repertoires (<jats:italic>P<\/jats:italic>\u2003=\u20030.575) for individual patients but significant differences in expansions\/contractions between CD4<jats:sup>+<\/jats:sup>CD25<jats:sup>Bright<\/jats:sup> and CD8<jats:sup>+<\/jats:sup> T lymphocytes repertoires (<jats:italic>P<\/jats:italic>\u2003=\u20030.011). There was bias in V\u03b2 usage between CD3<jats:sup>+<\/jats:sup>CD4<jats:sup>+<\/jats:sup> and CD3<jats:sup>+<\/jats:sup>CD4<jats:sup>\u2212<\/jats:sup> T lymphocyte subsets. A total of 67% patients had TCR V\u03b2 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.<\/jats:p>","DOI":"10.1111\/j.1365-3083.2011.02527.x","type":"journal-article","created":{"date-parts":[[2011,2,15]],"date-time":"2011-02-15T21:18:14Z","timestamp":1297804694000},"page":"577-585","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Flow Cytometric Analysis of TCR V\u03b2 Repertoire in Patients with 22q11.2 Deletion Syndrome"],"prefix":"10.1111","volume":"73","author":[{"given":"A.","family":"McLean\u2010Tooke","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Barge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. P.","family":"Spickett","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. 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