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Blood, 15 December 2004, Vol. 104, No. 13, pp. 3927-3935. Prepublished online as a Blood First Edition Paper on August 19, 2004; DOI 10.1182/blood-2003-10-3626. This Article Full Text (PDF) Supplemental Table and Figures All Versions of this Article: 2003-10-3626v1 104/13/3927    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 Franco, S. Articles by Blasco, M. A Search for Related Content PubMed PubMed Citation Articles by Franco, S. Articles by Blasco, M. A Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted November 14, 2003 Accepted August 5, 2004 Telomere dynamics in fancg-deficient mouse and human cells Sonia Franco, Henri J van de Vrugt, Piedad Fernandez, Miguel Aracil, Fre Arwert, and Maria A Blasco* Molecular Oncology Program, Spanish National Cancer Centre, Madrid, Spain Dpt. of Clinical Genetics and Human Genetics, VU University Medical Center, Amsterdam, The Netherlands Dpt. of Immunology and Oncology (DIO), National Center of Biotechnology (CNB-CSIC), Campus de Cantoblanco, Madrid, Spain * Corresponding author; email: mblasco{at}cnio.es. A number of DNA repair proteins also play roles in telomere metabolism. To investigate whether the accelerated telomere shortening reported in Fanconi anemia (FA) hematopoietic cells relates to a direct role of the FA pathway in telomere maintenance, we have analyzed telomere dynamics in Fancg-deficient mouse and human cells. We show here that both hematopoietic (stem and differentiated bone marrow cells, B and T lymphocytes) and non-hematopoietic (germ cells, MEFs) Fancg-/- mouse cells display normal telomere length, normal telomerase activity and normal chromosome end-capping, even in the presence of extensive clastogen-induced cytogenetic instability (MMC, gamma-radiation). In addition, telomerase-deficient MEFs with human-like telomere length and decreased Fancg expression (G5 Terc-/-/Fancg shRNA3 MEFs) display normal telomere maintenance. Finally, early-passage primary fibroblasts from FA patients of complementation group G as well as primary human cells with reduced FANCG expression (FANCG shRNA IMR-90 cells) show no signs of telomere dysfunction. Our observations indicate that accelerated telomere shortening in FA patients is not due to a role of FANCG at telomeres, but instead may be secondary to the disease. These findings suggest that telomerase-based therapies could be useful prophylactic agents in FA aplastic anemia, by preserving their telomere reserve in the context of the disease. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: Q. Fan, F. Zhang, B. Barrett, K. Ren, and P. R. Andreassen A role for monoubiquitinated FANCD2 at telomeres in ALT cells Nucleic Acids Res., April 1, 2009; 37(6): 1740 - 1754. [Abstract] [Full Text] [PDF] N. Spardy, A. Duensing, E. E. Hoskins, S. I. Wells, and S. Duensing HPV-16 E7 Reveals a Link between DNA Replication Stress, Fanconi Anemia D2 Protein, and Alternative Lengthening of Telomere-Associated Promyelocytic Leukemia Bodies Cancer Res., December 1, 2008; 68(23): 9954 - 9963. [Abstract] [Full Text] [PDF]

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