SEARCH:   Advanced

Blood, 15 July 2004, Vol. 104, No. 2, pp. 572-578. Prepublished online as a Blood First Edition Paper on March 25, 2004; DOI 10.1182/blood-2003-12-4226. This Article Full Text (PDF) All Versions of this Article: 2003-12-4226v1 104/2/572    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 Bagley, J. Articles by Iacomini, J. Search for Related Content PubMed PubMed Citation Articles by Bagley, J. Articles by Iacomini, J. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted December 11, 2003 Accepted March 2, 2004 Bone marrow transplantation restores immune system function and prevents lymphoma in Atm deficient mice Jessamyn Bagley, Maria L Cortes, Xandra O Breakefield, and John Iacomini* Transplantation Biology Research Center, Massachusetts General Hospital, Boston, MA, USA Molecular Neurogenetics Unit, Department of Neurology, Massachusetts General Hospital, Boston, MA, USA * Corresponding author; email: john.iacomini{at}tbrc.mgh.harvard.edu. Ataxia-telangiectasia (A-T) is a human autosomal recessive disease caused by mutations in the gene encoding ataxia-telangiectasia mutated (ATM). A-T is characterized by progressive cerebellar degeneration, variable immunodeficiency, and a high incidence of leukemia and lymphoma. Recurrent sino-pulmonary infections secondary to immunodeficiency and hematopoietic malignancies are major causes of morbidity and mortality in A-T patients. In mice, an introduced mutation in Atm leads to a phenotype that recapitulates many of the symptoms of A-T including immune system abnormalities and susceptibility to malignancy. Here we show that the replacement of the bone marrow compartment in Atm knockout mice (Atm-/-) using a clinically relevant, non-myeloablative host conditioning regimen can be used to overcome the immune deficiencies and prevent the malignancies observed in these mice. Therefore, bone marrow transplantation may prove to be of therapeutic benefit in A-T patients. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: J. Bagley, G. Singh, and J. Iacomini Regulation of Oxidative Stress Responses by Ataxia-Telangiectasia Mutated Is Required for T Cell Proliferation J. Immunol., April 15, 2007; 178(8): 4757 - 4763. [Abstract] [Full Text] [PDF] A. R. Gennery Primary immunodeficiency syndromes associated with defective DNA double-strand break repair Br. Med. Bull., October 5, 2006; (2006) ldl006v2. [Abstract] [Full Text] [PDF]

	Copyright © 2004 by American Society of Hematology         Online ISSN: 1528-0020