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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 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 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 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 Hemostasis, Thrombosis, and Vascular Biology Neoplasia Red Cells Review Articles Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  REVIEW ARTICLES Molecular pathogenesis of Fanconi anemia: recent progress Toshiyasu Taniguchi, and Alan D. D'Andrea From the Division of Human Biology and the Division of Public Health Sciences, Fred Hutchinson Cancer Research Center, Seattle, WA; and the Department of Radiation Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA. 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 or leukemia susceptibility, and cellular hypersensitivity to DNAcrosslinking agents. Identification of 11 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 anticancer 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: G. Zhi, J. B. Wilson, X. Chen, D. S. Krause, Y. Xiao, N. J. Jones, and G. M. Kupfer Fanconi Anemia Complementation Group FANCD2 Protein Serine 331 Phosphorylation Is Important for Fanconi Anemia Pathway Function and BRCA2 Interaction Cancer Res., November 15, 2009; 69(22): 8775 - 8783. [Abstract] [Full Text] [PDF] N. G. Howlett, J. A. Harney, M. A. Rego, F. W. Kolling IV, and T. W. 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Pommier DNA Repair Modulators as Anticancer Agents ASCO Educational Book, January 1, 2008; 2008(1): 128 - 133. [Abstract] [Full Text] [PDF] J. Kenyon and S. L. Gerson The role of DNA damage repair in aging of adult stem cells Nucleic Acids Res., December 26, 2007; (2007) gkm1064v1. [Abstract] [Full Text] [PDF] J. Grillari, H. Katinger, and R. Voglauer Contributions of DNA interstrand cross-links to aging of cells and organisms Nucleic Acids Res., December 14, 2007; (2007) gkm1065v1. [Abstract] [Full Text] [PDF] M. Berwick, J. M. Satagopan, L. Ben-Porat, A. Carlson, K. Mah, R. Henry, R. Diotti, K. Milton, K. Pujara, T. Landers, et al. Genetic Heterogeneity among Fanconi Anemia Heterozygotes and Risk of Cancer Cancer Res., October 1, 2007; 67(19): 9591 - 9596. [Abstract] [Full Text] [PDF] C. Jacquemont and T. Taniguchi Proteasome Function Is Required for DNA Damage Response and Fanconi Anemia Pathway Activation Cancer Res., August 1, 2007; 67(15): 7395 - 7405. [Abstract] [Full Text] [PDF] T. Oda, T. Hayano, H. Miyaso, N. Takahashi, and T. Yamashita Hsp90 regulates the Fanconi anemia DNA damage response pathway Blood, June 1, 2007; 109(11): 5016 - 5026. [Abstract] [Full Text] [PDF] N. Rahman and R. H. Scott Cancer genes associated with phenotypes in monoallelic and biallelic mutation carriers: new lessons from old players Hum. Mol. Genet., April 15, 2007; 16(R1): R60 - R66. [Abstract] [Full Text] [PDF] J. Antonio Casado, E. Callen, A. Jacome, P. Rio, M. Castella, S. Lobitz, T. Ferro, A. Munoz, J. Sevilla, A. Cantalejo, et al. A comprehensive strategy for the subtyping of patients with Fanconi anaemia: conclusions from the Spanish Fanconi Anemia Research Network J. Med. Genet., April 1, 2007; 44(4): 241 - 249. [Abstract] [Full Text] [PDF] R. K. Nookala, S. Hussain, and L. Pellegrini Insights into Fanconi Anaemia from the structure of human FANCE Nucleic Acids Res., March 12, 2007; 35(5): 1638 - 1648. [Abstract] [Full Text] [PDF] E. 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