{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T00:39:10Z","timestamp":1780101550663,"version":"3.54.0"},"reference-count":32,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2004,3,5]],"date-time":"2004-03-05T00:00:00Z","timestamp":1078444800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,3,5]]},"abstract":"<jats:title>SUMMARY<\/jats:title><jats:p>Wiskott\u2013Aldrich syndrome (WAS) is an X-linked platelet\/immunodeficiency disease. The affected gene encodes WASP, a multidomain protein that regulates cytoskeletal assembly in blood cells. Patients have recurring infections, and their lymphocytes exhibit deficient proliferative responses in vitro. We report an evaluation of peripheral blood lymphocytes of 27 WAS patients, aged one month to 55\u2003years. Whereas NK cells were normal, a significant deficit of T and B lymphocytes was observed. The number of lymphocytes was already decreased in infant patients, suggesting deficient output. Both CD4 and CD8 T lymphocytes were affected; the decrease was most pronounced for na\u00efve T cells. Na\u00efve CD4 lymphocytes of patients showed normal expression of Bcl-2, and Ki-67, and normal survival in vitro, suggesting that their in vivo survival and proliferation are normal. The collective data suggest that the patients\u2019 lymphocyte deficit results from deficient output, likely due to abnormal lymphocyte maturation in the thymus and bone marrow. We propose that WASP plays an important role not only in the function of mature T lymphocytes, but also in the maturation of human T and B lymphocytes and that impaired lymphocyte maturation is central to the aetiology of WAS immunodeficiency.<\/jats:p>","DOI":"10.1111\/j.1365-2249.2004.02409.x","type":"journal-article","created":{"date-parts":[[2004,3,18]],"date-time":"2004-03-18T10:17:18Z","timestamp":1079605038000},"page":"104-110","source":"Crossref","is-referenced-by-count":85,"title":["Early deficit of lymphocytes in Wiskott\u2013Aldrich syndrome: possible role of WASP in human lymphocyte maturation"],"prefix":"10.1093","volume":"136","author":[{"given":"J Y","family":"PARK","sequence":"first","affiliation":[{"name":"The CBR Institute for Biomedical Research"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"KOB","sequence":"additional","affiliation":[{"name":"The CBR Institute for Biomedical Research"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A P","family":"PRODEUS","sequence":"additional","affiliation":[{"name":"Research Institute for Paediatric Haematology, Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F S","family":"ROSEN","sequence":"additional","affiliation":[{"name":"The CBR Institute for Biomedical Research"},{"name":"Department of Pediatrics, Harvard Medical School, BostonUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"SHCHERBINA","sequence":"additional","affiliation":[{"name":"The CBR Institute for Biomedical Research"},{"name":"Research Institute for Paediatric Haematology, Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"REMOLD-O'DONNELL","sequence":"additional","affiliation":[{"name":"The CBR Institute for Biomedical Research"},{"name":"Department of Pediatrics, Harvard Medical School, BostonUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2004,3,18]]},"reference":[{"key":"2022011223182771800_b1","first-page":"212","article-title":"Familiarer, angeobren Morbus Werlhofi?","volume":"68","author":"Wiskott","year":"1937","journal-title":"Monatschrift Kinderheil"},{"key":"2022011223182771800_b2","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1542\/peds.13.2.133","article-title":"Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and blood diarrhea","volume":"13","author":"Aldrich","year":"1954","journal-title":"Pediatrics"},{"key":"2022011223182771800_b3","doi-asserted-by":"crossref","first-page":"4383","DOI":"10.4049\/jimmunol.151.8.4383","article-title":"T cells of patients with the Wiskott\u2013Aldrich syndrome have a restricted defect in proliferative responses","volume":"151","author":"Molina","year":"1993","journal-title":"J 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