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Blood, 15 September 2006, Vol. 108, No. 6, pp. 2072-2080. Prepublished online as a Blood First Edition Paper on May 23, 2006; DOI 10.1182/blood-2005-11-008151. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2005-11-008151v1 108/6/2072    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 Medhurst, A. L. Articles by de Winter, J. P. Search for Related Content PubMed PubMed Citation Articles by Medhurst, A. L. Articles by de Winter, J. P. Related Collections Hematopoiesis and Stem Cells Red Cells Signal Transduction Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  RED CELLS Evidence for subcomplexes in the Fanconi anemia pathway Annette L. Medhurst, El Houari Laghmani, Jurgen Steltenpool, Miriam Ferrer, Chantal Fontaine, Jan de Groot, Martin A. Rooimans, Rik J. Scheper, Amom Ruhikanta Meetei, Weidong Wang, Hans Joenje, and Johan P. de Winter From the Department of Clinical Genetics and Human Genetics, the Department of Medical Oncology, Division of Gene Therapy, and the Department of Pathology, Vrije Universiteit (VU) Medical Center, Amsterdam, The Netherlands; the Division of Experimental Hematology, Cincinnati Children's Research Foundation and University of Cincinnati College of Medicine, OH; and the Laboratory of Genetics, National Institute of Aging, National Institutes of Health (NIH), Baltimore, MD. Fanconi anemia (FA) is a genomic instability disorder, clinically characterized by congenital abnormalities, progressive bone marrow failure, and predisposition to malignancy. Cells derived from patients with FA display a marked sensitivity to DNA cross-linking agents, such as mitomycin C (MMC). This observation has led to the hypothesis that the proteins defective in FA are involved in the sensing or repair of interstrand cross-link lesions of the DNA. A nuclear complex consisting of a majority of the FA proteins plays a crucial role in this process and is required for the monoubiquitination of a downstream target, FANCD2. Two new FA genes, FANCB and FANCL, have recently been identified, and their discovery has allowed a more detailed study into the molecular architecture of the FA pathway. We demonstrate a direct interaction between FANCB and FANCL and that a complex of these proteins binds FANCA. The interaction between FANCA and FANCL is dependent on FANCB, FANCG, and FANCM, but independent of FANCC, FANCE, and FANCF. These findings provide a framework for the protein interactions that occur "upstream" in the FA pathway and suggest that besides the FA core complex different subcomplexes exist that may have specific functions other than the monoubiquitination of FANCD2. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Hsp90 regulates the Fanconi anemia DNA damage response pathway Tsukasa Oda, Toshiya Hayano, Hidenobu Miyaso, Nobuhiro Takahashi, and Takayuki Yamashita Blood 2007 109: 5016-5026. [Abstract] [Full Text] [PDF] This article has been cited by other articles: J. M. Kim, Y. Kee, A. Gurtan, and A. D. D'Andrea Cell cycle-dependent chromatin loading of the Fanconi anemia core complex by FANCM/FAAP24 Blood, May 15, 2008; 111(10): 5215 - 5222. [Abstract] [Full Text] [PDF] A. Alpi, F. Langevin, G. Mosedale, Y. J. Machida, A. Dutta, and K. J. Patel UBE2T, the Fanconi Anemia Core Complex, and FANCD2 Are Recruited Independently to Chromatin: a Basis for the Regulation of FANCD2 Monoubiquitination Mol. Cell. Biol., December 15, 2007; 27(24): 8421 - 8430. [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] 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] S. S. Mukhopadhyay, K. S. Leung, M. J. Hicks, P. J. Hastings, H. Youssoufian, and S. E. Plon Defective mitochondrial peroxiredoxin-3 results in sensitivity to oxidative stress in Fanconi anemia J. Cell Biol., October 23, 2006; 175(2): 225 - 235. [Abstract] [Full Text] [PDF]

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