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Prepublished online as a Blood First Edition Paper on March 6, 2003; DOI 10.1182/blood-2003-01-0128. This Article Full Text (PDF) All Versions of this Article: 2003-01-0128v1 102/2/514    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 Karadimitris, A. Articles by Notaro, R. Search for Related Content PubMed PubMed Citation Articles by Karadimitris, A. Articles by Notaro, R. Social Bookmarking                   What's this? Submitted January 17, 2003 Accepted February 24, 2003 Severe telomere shortening affects both GPI- and GPI+ hematopoiesis in patients with paroxysmal nocturnal hemoglobinuria Anastasios Karadimitris, David J Araten, Lucio Luzzatto*, and Rosario Notaro Department of Human Genetics and Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, NY, USA Department of Hematology, Imperial College School of Medicine, Hammersmith Hospital, London, United Kingdom Laboratorio di Genetica Umana, Dipartimento di Eziologia ed Epidemiologia, IST, Istituto Nazionale per la Ricerca sul Cancro, Genova, Italy * Corresponding author; email: lucio.luzzatto{at}istge.it. A most distinctive feature of paroxysmal nocturnal hemoglobinuria (PNH) is that in each patient GPI- and GPI+ hematopoietic stem cells (HSC) co-exist, and both contribute to hematopoiesis. Telomere size correlates inversely with the cell division history of HSC. In 10 patients with hemolytic PNH the telomeres in sorted GPI- granulocytes were shorter than in sorted GPI+ granulocytes in 4 cases, comparable in 2 cases, and longer in the remaining 4 cases. Furthermore, the telomeres of both GPI- and GPI+ hematopoietic cells were markedly shortened compared to age-matched controls. The short telomeres in the GPI- cells probably reflect the large number of cell divisions required for the progeny of a single cell to contribute a large proportion of hematopoiesis. The short telomeres of the GPI+ cells indicate that the residual hematopoiesis contributed by these cells is not normal. This epigenetic change is an additional feature shared by PNH and aplastic anemia. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: L. Mollica, I. Fleury, C. Belisle, S. Provost, D.-C. Roy, and L. Busque No Association Between Telomere Length and Blood Cell Counts in Elderly Individuals J Gerontol A Biol Sci Med Sci, September 1, 2009; 64A(9): 965 - 967. [Abstract] [Full Text] [PDF] C. K. Garcia, W. E. Wright, and J. W. Shay Human diseases of telomerase dysfunction: insights into tissue aging Nucleic Acids Res., December 3, 2007; 35(22): 7406 - 7416. [Abstract] [Full Text] [PDF] B. P. Alter, G. M. Baerlocher, S. A. Savage, S. J. Chanock, B. B. Weksler, J. P. Willner, J. A. Peters, N. Giri, and P. M. Lansdorp Very short telomere length by flow fluorescence in situ hybridization identifies patients with dyskeratosis congenita Blood, September 1, 2007; 110(5): 1439 - 1447. [Abstract] [Full Text] [PDF] K. M. Austin, R. J. Leary, and A. Shimamura The Shwachman-Diamond SBDS protein localizes to the nucleolus Blood, August 15, 2005; 106(4): 1253 - 1258. [Abstract] [Full Text] [PDF] F. Beier, S. Balabanov, T. Buckley, K. Dietz, U. Hartmann, M. Rojewski, L. Kanz, H. Schrezenmeier, and T. H. Brummendorf Accelerated telomere shortening in glycosylphosphatidylinositol (GPI)-negative compared with GPI-positive granulocytes from patients with paroxysmal nocturnal hemoglobinuria (PNH) detected by proaerolysin flow-FISH Blood, July 15, 2005; 106(2): 531 - 533. [Abstract] [Full Text] [PDF]

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