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Blood, 1 December 2005, Vol. 106, No. 12, pp. 3895-3897. Prepublished online as a Blood First Edition Paper on August 9, 2005; DOI 10.1182/blood-2005-06-2336. This Article Full Text (PDF) All Versions of this Article: 2005-06-2336v1 106/12/3895    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Wada, T. Articles by Candotti, F. Search for Related Content PubMed PubMed Citation Articles by Wada, T. Articles by Candotti, F. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted June 13, 2005 Accepted August 2, 2005 Analysis of T-cell repertoire diversity in Wiskott-Aldrich syndrome Taizo Wada, Shepherd H Schurman, Elizabeth K Garabedian, Akihiro Yachie, and Fabio Candotti* Genetics and Molecular Biology Branch and Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA Department of Laboratory Sciences, School of Health Sciences, Faculty of Medicine, Kanazawa University, Kanazawa, Japan * Corresponding author; email: fabio{at}nhgri.nih.gov. Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency characterized by thrombocytopenia, eczema, and variable degrees of impaired cellular and humoral immunity. Age-dependent T-cell lymphopenia has been described in WAS, however, the diversity of the T-cell compartment over time in these patients has not been characterized. We have used complementarity-determining region 3 (CDR3) size distribution analysis to assess T-cell receptor (TCR) V repertoire in 13 WAS patients. Diverse CDR3 size pattern was demonstrated in patients under 15 years of age regardless of the levels of WAS protein (WASP) expression. In contrast, older patients showed significantly higher skewing of TCRV repertoire as compared with healthy adults. We did not find correlation between clinical score and complexity of TCRV repertoire. These findings suggest that WASP deficiency does not limit thymic generation of a normal TCR and indicate that T-cell oligoclonality may contribute to the immunodeficiency in older WAS patients. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Bosticardo, F. Marangoni, A. Aiuti, A. Villa, and M. Grazia Roncarolo Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome Blood, June 18, 2009; 113(25): 6288 - 6295. [Abstract] [Full Text] [PDF] S.-J. Lin, A. T. Chen, and R. M. Welsh Immune system derived from homeostatic proliferation generates normal CD8 T-cell memory but altered repertoires and diminished heterologous immune responses Blood, August 1, 2008; 112(3): 680 - 689. [Abstract] [Full Text] [PDF] F. Marangoni, S. Trifari, S. Scaramuzza, C. Panaroni, S. Martino, L. D. Notarangelo, Z. Baz, A. Metin, F. Cattaneo, A. Villa, et al. WASP regulates suppressor activity of human and murine CD4+CD25+FOXP3+ natural regulatory T cells J. Exp. Med., February 19, 2007; 204(2): 369 - 380. [Abstract] [Full Text] [PDF] S. Trifari, G. Sitia, A. Aiuti, S. Scaramuzza, F. Marangoni, L. G. Guidotti, S. Martino, P. Saracco, L. D. Notarangelo, M.-G. Roncarolo, et al. Defective Th1 Cytokine Gene Transcription in CD4+ and CD8+ T Cells from Wiskott-Aldrich Syndrome Patients J. Immunol., November 15, 2006; 177(10): 7451 - 7461. [Abstract] [Full Text] [PDF]

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