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Blood, 1 June 2006, Vol. 107, No. 11, pp. 4223-4233. Prepublished online as a Blood First Edition Paper on February 21, 2006; DOI 10.1182/blood-2005-10-4240. This Article Full Text (PDF) All Versions of this Article: 2005-10-4240v1 107/11/4223    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 Taniguchi, T. Articles by D'Andrea, A. D Search for Related Content PubMed PubMed Citation Articles by Taniguchi, T. Articles by D'Andrea, A. D Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted October 26, 2005 Accepted January 21, 2006 The molecular pathogenesis of fanconi anemia: recent progress Toshiyasu Taniguchi and Alan D D'Andrea* Divisions of Human Biology and Public Health Sciences, Fred Hutchinson Cancer Research Center, Seattle, WA, USA Department of Radiation Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA * Corresponding author; email: alan_dandrea{at}dfci.harvard.edu. A rare genetic disease, Fanconi Anemia (FA), now attracts broader attention from cancer biologists and basic researchers in the DNA repair and ubiquitin biology fields as well as from hematologists. FA is a chromosome instability syndrome characterized by childhood-onset aplastic anemia, cancer/leukemia susceptibility, and cellular hypersensitivity to DNA crosslinking agents. Identification of eleven genes for FA has led to progress in the molecular understanding of this disease. FA proteins, including a ubiquitin ligase (FANCL), a monoubiquitinated protein (FANCD2), a helicase (FANCJ/BACH1/BRIP1) and a breast/ovarian cancer susceptibility protein (FANCD1/BRCA2), appear to cooperate in a pathway leading to the recognition and repair of damaged DNA. Molecular interactions among FA proteins and responsible proteins for other chromosome instability syndromes (BLM, NBS1, MRE11, ATM, and ATR) have also been found. Furthermore, inactivation of FA genes has been observed in a wide variety of human cancers in the general population. These findings have broad implications for predicting the sensitivity and resistance of tumors to widely-used anti-cancer DNA crosslinking agents (cisplatin, mitomycin C, and melphalan). Here, we summarize recent progress in the molecular biology of FA and discuss roles of the FA proteins in DNA repair and cancer biology. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: The less-traveled Fanconi road Grover C. Bagby Blood 2006 107: 4196-4197. [Full Text] [PDF] This article has been cited by other articles: A. De Nicolo, M. Tancredi, G. Lombardi, C. C. Flemma, S. Barbuti, C. Di Cristofano, B. Sobhian, G. Bevilacqua, R. Drapkin, and M. A. Caligo A Novel Breast Cancer-Associated BRIP1 (FANCJ/BACH1) Germ-line Mutation Impairs Protein Stability and Function Clin. Cancer Res., July 15, 2008; 14(14): 4672 - 4680. [Abstract] [Full Text] [PDF] J. A. Casado, P. Rio, E. Marco, V. Garcia-Hernandez, A. Domingo, L. Perez, J. C. Tercero, J. J. Vaquero, B. Albella, F. Gago, et al. Relevance of the Fanconi anemia pathway in the response of human cells to trabectedin Mol. Cancer Ther., May 1, 2008; 7(5): 1309 - 1318. [Abstract] [Full Text] [PDF] R. T. Calado and N. S. 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