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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 (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 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 Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted November 10, 2005 Accepted May 7, 2006 Evidence for subcomplexes in the Fanconi anaemia 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* Dept of Clinical Genetics and Human Genetics, VU Medical Center, Amsterdam, The Netherlands Dept of Medical Oncology, Division of Gene Therapy, VU Medical Center, Amsterdam, The Netherlands Dept of Pathology VU Medical Center, Amsterdam, The Netherlands Div Experimental Hematology, Cincinnati Children's Research Foundation, Cincinnati, Ohio, USA 5Laboratory of Genetics, National Institute of Aging, NIH, Baltimore, Maryland, USA * Corresponding author; email: j.dewinter{at}vumc.nl. Fanconi anaemia (FA) is a genomic instability disorder, clinically characterised by congenital abnormalities, progressive bone marrow failure and predisposition to malignancy. Cells derived from FA patients 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: S. T. Bakker, H. J. van de Vrugt, M. A. Rooimans, A. B. Oostra, J. Steltenpool, E. Delzenne-Goette, A. van der Wal, M. van der Valk, H. Joenje, H. te Riele, et al. Fancm-deficient mice reveal unique features of Fanconi anemia complementation group M Hum. Mol. Genet., September 15, 2009; 18(18): 3484 - 3495. [Abstract] [Full Text] [PDF] T. R. Singh, S. T. Bakker, S. Agarwal, M. Jansen, E. Grassman, B. C. Godthelp, A. M. Ali, C.-h. Du, M. A. Rooimans, Q. Fan, et al. Impaired FANCD2 monoubiquitination and hypersensitivity to camptothecin uniquely characterize Fanconi anemia complementation group M Blood, July 2, 2009; 114(1): 174 - 180. [Abstract] [Full Text] [PDF] Y. Kee, J. M. Kim, and A. D'Andrea Regulated degradation of FANCM in the Fanconi anemia pathway during mitosis Genes & Dev., March 1, 2009; 23(5): 555 - 560. [Abstract] [Full Text] [PDF] 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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