SEARCH:   Advanced

Blood, 15 January 2004, Vol. 103, No. 2, pp. 399-406. Prepublished online as a Blood First Edition Paper on September 25, 2003; DOI 10.1182/blood-2003-05-1556. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-05-1556v1 103/2/399    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map 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 Gurbuxani, S. Articles by Crispino, J. D. Search for Related Content PubMed PubMed Citation Articles by Gurbuxani, S. Articles by Crispino, J. D. Related Collections Hematopoiesis and Stem Cells Immunobiology Apoptosis Gene Expression Reviews in Translational Hematology Related Letter in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  REVIEWS IN TRANSLATIONAL HEMATOLOGY Recent insights into the mechanisms of myeloid leukemogenesis in Down syndrome Sandeep Gurbuxani, Paresh Vyas, and John D. Crispino From the Ben May Institute for Cancer Research, University of Chicago, Chicago, IL; Department of Haematology, University of Oxford, Weatherall Institute for Molecular Medicine, John Radcliffe Hospital, Oxford, United Kingdom. GATA-1 is the founding member of a transcription factor family that regulates growth and maturation of a diverse set of tissues. GATA-1 is expressed primarily in hematopoietic cells and is essential for proper development of erythroid cells, megakaryocytes, eosinophils, and mast cells. Although loss of GATA-1 leads to differentiation arrest and apoptosis of erythroid progenitors, absence of GATA-1 promotes accumulation of immature megakaryocytes. Recently, we and others have reported that mutagenesis of GATA1 is an early event in Down syndrome (DS) leukemogenesis. Acquired mutations in GATA1 were detected in the vast majority of patients with acute megakaryoblastic leukemia (DS-AMKL) and in nearly every patient with transient myeloproliferative disorder (TMD), a "preleukemia" that may be present in as many as 10% of infants with DS. Although the precise pathway by which mutagenesis of GATA1 contributes to leukemia is unknown, these findings confirm that GATA1 plays an important role in both normal and malignant hematopoiesis. Future studies to define the mechanism that results in the high frequency of GATA1 mutations in DS and the role of altered GATA1 in TMD and DS-AMKL will shed light on the multistep pathway in human leukemia and may lead to an increased understanding of why children with DS are markedly predisposed to leukemia. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Letter in Blood Online: Prenatal origin of GATA1 mutations may be an initiating step in the development of megakaryocytic leukemia in Down syndrome Jeffrey W. Taub, Gina Mundschau, Yubin Ge, Janet M. Poulik, Faisal Qureshi, Tanya Jensen, S. Jill James, Larry H. Matherly, Joshua Wechsler, and John D. Crispino Blood 2004 104: 1588-1589. [Full Text] [PDF] This article has been cited by other articles: J. W. Vardiman, J. Thiele, D. A. Arber, R. D. Brunning, M. J. Borowitz, A. Porwit, N. L. Harris, M. M. Le Beau, E. Hellstrom-Lindberg, A. Tefferi, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes Blood, July 30, 2009; 114(5): 937 - 951. [Abstract] [Full Text] [PDF] M. J. Stankiewicz and J. D. Crispino ETS2 and ERG promote megakaryopoiesis and synergize with alterations in GATA-1 to immortalize hematopoietic progenitor cells Blood, April 2, 2009; 113(14): 3337 - 3347. [Abstract] [Full Text] [PDF] K. R. Rabin and J. A. Whitlock Malignancy in Children with Trisomy 21 Oncologist, February 1, 2009; 14(2): 164 - 173. [Abstract] [Full Text] [PDF] S. T. Chou, J. B. Opalinska, Y. Yao, M. A. Fernandes, A. Kalota, J. S. J. Brooks, J. K. Choi, A. M. Gewirtz, G.-a. Danet-Desnoyers, R. L. Nemiroff, et al. Trisomy 21 enhances human fetal erythro-megakaryocytic development Blood, December 1, 2008; 112(12): 4503 - 4506. [Abstract] [Full Text] [PDF] T. Yokomizo, K. Hasegawa, H. Ishitobi, M. Osato, M. Ema, Y. Ito, M. Yamamoto, and S. Takahashi Runx1 is involved in primitive erythropoiesis in the mouse Blood, April 15, 2008; 111(8): 4075 - 4080. [Abstract] [Full Text] [PDF] G. Kirsammer, S. Jilani, H. Liu, E. Davis, S. Gurbuxani, M. M. Le Beau, and J. D. Crispino Highly penetrant myeloproliferative disease in the Ts65Dn mouse model of Down syndrome Blood, January 15, 2008; 111(2): 767 - 775. [Abstract] [Full Text] [PDF] A. Norton, C. Fisher, H. Liu, Q. Wen, G. Mundschau, J. L. Fuster, H. Hasle, B. Zeller, D. K. Webb, A. O'Marcaigh, et al. Analysis of JAK3, JAK2, and C-MPL mutations in transient myeloproliferative disorder and myeloid leukemia of Down syndrome blasts in children with Down syndrome Blood, August 1, 2007; 110(3): 1077 - 1079. [Full Text] [PDF] S. Malinge, R. Ben-Abdelali, C. Settegrana, I. Radford-Weiss, M. Debre, K. Beldjord, E. A. Macintyre, J.-L. Villeval, W. Vainchenker, R. Berger, et al. Novel activating JAK2 mutation in a patient with Down syndrome and B-cell precursor acute lymphoblastic leukemia Blood, March 1, 2007; 109(5): 2202 - 2204. [Abstract] [Full Text] [PDF] J. Tober, A. Koniski, K. E. McGrath, R. Vemishetti, R. Emerson, K. K. L. de Mesy-Bentley, R. Waugh, and J. Palis The megakaryocyte lineage originates from hemangioblast precursors and is an integral component both of primitive and of definitive hematopoiesis Blood, February 15, 2007; 109(4): 1433 - 1441. [Abstract] [Full Text] [PDF] A. M. Linabery, A. F. Olshan, A. S. Gamis, F. O. Smith, N. A. Heerema, C. K. Blair, and J. A. Ross Exposure to Medical Test Irradiation and Acute Leukemia Among Children With Down Syndrome: A Report From the Children's Oncology Group Pediatrics, November 1, 2006; 118(5): e1499 - e1508. [Abstract] [Full Text] [PDF] T. Mercher, G. Wernig, S. A. Moore, R. L. Levine, T.-L. Gu, S. Frohling, D. Cullen, R. D. Polakiewicz, O. A. Bernard, T. J. Boggon, et al. JAK2T875N is a novel activating mutation that results in myeloproliferative disease with features of megakaryoblastic leukemia in a murine bone marrow transplantation model Blood, October 15, 2006; 108(8): 2770 - 2779. [Abstract] [Full Text] [PDF] M. Pellegrini-Calace and A. Tramontano Identification of a novel putative mitogen-activated kinase cascade on human chromosome 21 by computational approaches Bioinformatics, April 1, 2006; 22(7): 775 - 778. [Abstract] [Full Text] [PDF] J.-P. Bourquin, A. Subramanian, C. Langebrake, D. Reinhardt, O. Bernard, P. Ballerini, A. Baruchel, H. Cave, N. Dastugue, H. Hasle, et al. Identification of distinct molecular phenotypes in acute megakaryoblastic leukemia by gene expression profiling PNAS, February 28, 2006; 103(9): 3339 - 3344. [Abstract] [Full Text] [PDF] W. Zhao, C. Kitidis, M. D. Fleming, H. F. Lodish, and S. Ghaffari Erythropoietin stimulates phosphorylation and activation of GATA-1 via the PI3-kinase/AKT signaling pathway Blood, February 1, 2006; 107(3): 907 - 915. [Abstract] [Full Text] [PDF] D. L. Stachura, S. T. Chou, and M. J. Weiss Early block to erythromegakaryocytic development conferred by loss of transcription factor GATA-1 Blood, January 1, 2006; 107(1): 87 - 97. [Abstract] [Full Text] [PDF] C. Kuhl, A. Atzberger, F. Iborra, B. Nieswandt, C. Porcher, and P. Vyas GATA1-Mediated Megakaryocyte Differentiation and Growth Control Can Be Uncoupled and Mapped to Different Domains in GATA1 Mol. Cell. Biol., October 1, 2005; 25(19): 8592 - 8606. [Abstract] [Full Text] [PDF] A. M. Vannucchi, A. Pancrazzi, P. Guglielmelli, S. Di Lollo, C. Bogani, G. Baroni, L. Bianchi, A. R. Migliaccio, A. Bosi, and F. Paoletti Abnormalities of GATA-1 in Megakaryocytes from Patients with Idiopathic Myelofibrosis Am. J. Pathol., September 1, 2005; 167(3): 849 - 858. [Abstract] [Full Text] [PDF] F. Rosenbauer, S. Koschmieder, U. Steidl, and D. G. Tenen Effect of transcription-factor concentrations on leukemic stem cells Blood, September 1, 2005; 106(5): 1519 - 1524. [Abstract] [Full Text] [PDF] A. G. Muntean and J. D. Crispino Differential requirements for the activation domain and FOG-interaction surface of GATA-1 in megakaryocyte gene expression and development Blood, August 15, 2005; 106(4): 1223 - 1231. [Abstract] [Full Text] [PDF] V. Munugalavadla, L. C. Dore, B. L. Tan, L. Hong, M. Vishnu, M. J. Weiss, and R. Kapur Repression of c-Kit and Its Downstream Substrates by GATA-1 Inhibits Cell Proliferation during Erythroid Maturation Mol. Cell. Biol., August 1, 2005; 25(15): 6747 - 6759. [Abstract] [Full Text] [PDF] X. Pan, O. Ohneda, K. Ohneda, F. Lindeboom, F. Iwata, R. Shimizu, M. Nagano, N. Suwabe, S. Philipsen, K.-C. Lim, et al. Graded Levels of GATA-1 Expression Modulate Survival, Proliferation, and Differentiation of Erythroid Progenitors J. Biol. Chem., June 10, 2005; 280(23): 22385 - 22394. [Abstract] [Full Text] [PDF] A. M. Vannucchi, L. Bianchi, F. Paoletti, A. Pancrazzi, E. Torre, M. Nishikawa, M. Zingariello, A. Di Baldassarre, R. A. Rana, R. Lorenzini, et al. A pathobiologic pathway linking thrombopoietin, GATA-1, and TGF-{beta}1 in the development of myelofibrosis Blood, May 1, 2005; 105(9): 3493 - 3501. [Abstract] [Full Text] [PDF] D. P. Steensma Down syndrome in Down House: trisomy 21, GATA1 mutations, and Charles Darwin Blood, March 15, 2005; 105(6): 2614 - 2616. [Full Text] [PDF] R. Ferreira, K. Ohneda, M. Yamamoto, and S. Philipsen GATA1 Function, a Paradigm for Transcription Factors in Hematopoiesis Mol. Cell. Biol., February 15, 2005; 25(4): 1215 - 1227. [Full Text] [PDF] M. Nakano, K. Ohneda, H. Yamamoto-Mukai, R. Shimizu, O. Ohneda, S. Ohmura, M. Suzuki, S. Tsukamoto, T. Yanagawa, H. Yoshida, et al. Transgenic over-expression of GATA-1 mutant lacking N-finger domain causes hemolytic syndrome in mouse erythroid cells Genes Cells, January 1, 2005; 10(1): 47 - 62. [Abstract] [Full Text] [PDF] R. Shimizu, T. Kuroha, O. Ohneda, X. Pan, K. Ohneda, S. Takahashi, S. Philipsen, and M. Yamamoto Leukemogenesis Caused by Incapacitated GATA-1 Function Mol. Cell. Biol., December 15, 2004; 24(24): 10814 - 10825. [Abstract] [Full Text] [PDF] J. W. Taub, G. Mundschau, Y. Ge, J. M. Poulik, F. Qureshi, T. Jensen, S. J. James, L. H. Matherly, J. Wechsler, and J. D. Crispino Prenatal origin of GATA1 mutations may be an initiating step in the development of megakaryocytic leukemia in Down syndrome Blood, September 1, 2004; 104(5): 1588 - 1589. [Full Text] [PDF]

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