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Blood, 1 June 2005, Vol. 105, No. 11, pp. 4523-4526. Prepublished online as a Blood First Edition Paper on February 10, 2005; DOI 10.1182/blood-2004-07-2762. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-07-2762v1 105/11/4523    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 Chan, E. M. Articles by Hromas, R. A. Search for Related Content PubMed PubMed Citation Articles by Chan, E. M. Articles by Hromas, R. A. Related Collections Hematopoiesis and Stem Cells Neoplasia Oncogenes and Tumor Suppressors Brief Reports Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  NEOPLASIA Brief report AML1-FOG2 fusion protein in myelodysplasia Edward M. Chan, Elisha M. Comer, Frank C. Brown, Kathleen E. Richkind, Melissa L. Holmes, Beng H. Chong, Roger Shiffman, Dong-Er Zhang, Marilyn L. Slovak, Cheryl L. Willman, Constance T. Noguchi, Yanjun Li, Devan J. Heiber, Lori Kwan, Rebecca J. Chan, Gail H. Vance, Heather C. Ramsey, and Robert A. Hromas From the Departments of Medicine, Pediatrics, and Medical & Molecular Genetics, Indiana University School of Medicine, and the Indiana University Cancer Center, Indianapolis, IN; Genzyme Genetics, Santa Fe, NM; Department of Medicine, University of New South Wales, Sydney, Australia; Monterey Bay Oncology Center, Monterey, CA; Scripps Research Institute, La Jolla, CA; City of Hope National Medical Center, Duarte, CA; National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD; Departments of Medicine and Pathology, and the Cancer Research & Treatment Center, University of New Mexico, Albuquerque, NM. Core binding factor (CBF) participates in specification of the hematopoietic stem cell and functions as a critical regulator of hematopoiesis. Translocation or point mutation of acute myeloid leukemia 1 (AML1)/RUNX1, which encodes the DNA-binding subunit of CBF, plays a central role in the pathogenesis of acute myeloid leukemia and myelodysplasia. We characterized the t(X;21)(p22.3;q22.1) in a patient with myelodysplasia that fuses AML1 in-frame to the novel partner gene FOG2/ZFPM2. The reciprocal gene fusions AML1-FOG2 and FOG2-AML1 are both expressed. AML1-FOG2, which fuses the DNA-binding domain of AML1 to most of FOG2, represses the transcriptional activity of both CBF and GATA1. AML1-FOG2 retains a motif that recruits the corepressor C-terminal binding protein (CtBP) and these proteins associate in a protein complex. These results suggest a central role for CtBP in AML1-FOG2 transcriptional repression and implicate coordinated disruption of the AML1 and GATAdevelopmental programs in the pathogenesis of myelodysplasia. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: L. F. Peterson, M. Yan, and D.-E. Zhang The p21Waf1 pathway is involved in blocking leukemogenesis by the t(8;21) fusion protein AML1-ETO Blood, May 15, 2007; 109(10): 4392 - 4398. [Abstract] [Full Text] [PDF] N. Asou, M. Yanagida, L. Huang, M. Yamamoto, K. Shigesada, H. Mitsuya, Y. Ito, and M. Osato Concurrent transcriptional deregulation of AML1/RUNX1 and GATA factors by the AML1-TRPS1 chimeric gene in t(8;21)(q24;q22) acute myeloid leukemia Blood, May 1, 2007; 109(9): 4023 - 4027. [Abstract] [Full Text] [PDF]

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