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Blood, 15 September 2006, Vol. 108, No. 6, pp. 1941-1948. Prepublished online as a Blood First Edition Paper on May 18, 2006; DOI 10.1182/blood-2006-04-019679. This Article Full Text (PDF) Supplemental Methods, Data, Table, and Figures All Versions of this Article: blood-2006-04-019679v1 108/6/1941    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 Hassler, S. Articles by Winqvist, O. Search for Related Content PubMed PubMed Citation Articles by Hassler, S. Articles by Winqvist, O. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted December 22, 2005 Accepted April 28, 2006 Aire deficient mice develop hematopoetic irregularities and marginal zone B cell lymphoma Signe Hassler, Chris Ramsey, Mikael C Karlsson, Disa Larsson, Bjorn Herrmann, Bjorn Rozell, Magnus Backheden, Leena Peltonen, Olle Kampe, and Ola Winqvist* Dept. of Medical Sciences, Uppsala University Hospital, Uppsala, Sweden Department of Human Genetics, David Geffen School of Medicine, Gonda Center, UCLA, CA, USA Karolinska Institutet,Dept. of Medicine,Unit of Clinical Allergy Research,Karolinska Hospital,Sweden Dept. of Medical Sciences, Section of Clinical Bacteriology, Uppsala University Hospital, Uppsala Karolinska Institutet,Division of Clinical Research Center & Pathology,Karolinska Hospital,Huddinge Dept. of Learning, Informatics, Management and Ethics (LIME), Karolinska Institutet,Stockholm,Sweden * Corresponding author; email: ola.winqvist{at}karolinska.se. Autoimmune polyendocrine syndrome type I (APS I) is an inherited recessive disorder with a progressive immunological destruction of many tissues including the adrenal cortex, the parathyroid glands and the gonads. APS I is caused by mutations in the AIRE gene (AutoImmune REgulator), expressed in cells of the thymus and spleen, suggesting a role in central and peripheral tolerance. Aire-/- mice replicate the autoimmune features of APS I patients with the presence of multiple autoantibodies and lymphocytic infiltrates in various tissues, but young mice appear clinically healthy. We here report the investigation of 15-24 months old Aire-/- mice. We did not observe any endocrinological abnormalities nor did sera from these mice recognize known APS I autoantigens. Interestingly, however, there was a high frequency of marginal zone B cell lymphoma in Aire-/- mice and liver infiltrates of B cells, suggesting chronic antigen exposure and exaggerated activation. Furthermore, increased numbers of monocytes in blood were identified as well as augmented numbers of metallophilic macrophages in the spleen. We propose that Aire, in addition to its function in the thymus, also has a peripheral regulatory role by controlling the development of APCs and marginal zone B cell activation. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. S. B. Wolff, M. M. Erichsen, A. Meager, N. F. Magitta, A. G. Myhre, J. Bollerslev, K. J. Fougner, K. Lima, P. M. Knappskog, and E. S. Husebye Autoimmune Polyendocrine Syndrome Type 1 in Norway: Phenotypic Variation, Autoantibodies, and Novel Mutations in the Autoimmune Regulator Gene J. Clin. Endocrinol. Metab., February 1, 2007; 92(2): 595 - 603. [Abstract] [Full Text] [PDF]

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