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Articles by Grompe, M Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Germ cell defects and hematopoietic hypersensitivity to gamma- interferon in mice with a targeted disruption of the Fanconi anemia C gene MA Whitney, G Royle, MJ Low, MA Kelly, MK Axthelm, C Reifsteck, S Olson, RE Braun, MC Heinrich, RK Rathbun, GC Bagby and M Grompe Department of Molecular and Medical Genetics, Vollum Institute, Oregon Regional Primate Center, Portland, USA Fanconi anemia (FA) is an autosomal recessive chromosome instability syndrome characterized by progressive bone marrow (BM) failure, skeletal defects, and increased susceptibility to malignancy. FA cells are hypersensitive to DNA cross-linking agents, oxygen and have cell cycle abnormalities. To develop an animal model of the disease we generated mice homozygous for a targeted deletion of exon 9 of the murine FA complementation group C gene (fac). Mutant mice had normal neonatal viability and gross morphology, but their cells had the expected chromosome breakage and DNA cross-linker sensitivity. Surprisingly, male and female mutant mice had reduced numbers of germ cells and females had markedly impaired fertility. No anemia was detectable in the peripheral blood during the first year of life, but the colony forming capacity of marrow progenitor cells was abnormal in vitro in mutant mice. Progenitor cells from fac knock-out mice were hypersensitive to interferon gamma. This previously unrecognized phenotype may form the basis for BM failure in human FA. Volume 88, Issue 1, pp. 49-58, 07/01/1996 Copyright © 1996 by The American Society of Hematology CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? 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. 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Bult The Mouse Tumor Biology Database: A Public Resource for Cancer Genetics and Pathology of the Mouse Cancer Res., March 1, 2002; 62(5): 1235 - 1240. [Abstract] [Full Text] [PDF] M. Koomen, N. C. Cheng, H. J. van de Vrugt, B. C. Godthelp, M. A. van der Valk, A. B. Oostra, M. Z. Zdzienicka, H. Joenje, and F. Arwert Reduced fertility and hypersensitivity to mitomycin C characterize Fancg/Xrcc9 null mice Hum. Mol. Genet., February 1, 2002; 11(3): 273 - 281. [Abstract] [Full Text] [PDF] Y. Yang, Y. Kuang, R. M. De Oca, T. Hays, L. Moreau, N. Lu, B. Seed, and A. D. D'Andrea Targeted disruption of the murine Fanconi anemia gene, Fancg/Xrcc9 Blood, December 1, 2001; 98(12): 3435 - 3440. [Abstract] [Full Text] [PDF] M. Grompe and A. D'Andrea Fanconi anemia and DNA repair Hum. Mol. Genet., October 1, 2001; 10(20): 2253 - 2259. [Abstract] [Full Text] [PDF] Q. Pang, T. A. Christianson, W. Keeble, J. Diaz, G. R. Faulkner, C. Reifsteck, S. Olson, and G. C. Bagby The Fanconi anemia complementation group C gene product: structural evidence of multifunctionality Blood, September 1, 2001; 98(5): 1392 - 1401. [Abstract] [Full Text] [PDF] S. Hadjur, K. Ung, L. Wadsworth, J. Dimmick, E. Rajcan-Separovic, R. W. Scott, M. Buchwald, and F. R. Jirik Defective hematopoiesis and hepatic steatosis in mice with combined deficiencies of the genes encoding Fancc and Cu/Zn superoxide dismutase Blood, August 15, 2001; 98(4): 1003 - 1011. [Abstract] [Full Text] [PDF] S. R. Fagerlie, J. Diaz, T. A. Christianson, K. McCartan, W. Keeble, G. R. Faulkner, and G. C. Bagby Functional correction of FA-C cells with FANCC suppresses the expression of interferon {gamma}-inducible genes Blood, May 15, 2001; 97(10): 3017 - 3024. [Abstract] [Full Text] [PDF] A. Zunino, P. Degan, T. Vigo, and A. Abbondandolo Hydrogen peroxide: effects on DNA, chromosomes, cell cycle and apoptosis induction in Fanconi's anemia cell lines Mutagenesis, May 1, 2001; 16(3): 283 - 288. [Abstract] [Full Text] [PDF] Q. Pang, W. Keeble, J. Diaz, T. A. Christianson, S. Fagerlie, K. Rathbun, G. R. Faulkner, M. O'Dwyer, and G. C. Bagby Jr Role of double-stranded RNA-dependent protein kinase in mediating hypersensitivity of Fanconi anemia complementation group C cells to interferon {gamma}, tumor necrosis factor-{alpha}, and double-stranded RNA Blood, March 15, 2001; 97(6): 1644 - 1652. [Abstract] [Full Text] [PDF] R. K. Rathbun, T. A. Christianson, G. R. Faulkner, G. Jones, W. Keeble, M. O'Dwyer, and G. C. Bagby Interferon-gamma -induced apoptotic responses of Fanconi anemia group C hematopoietic progenitor cells involve caspase 8-dependent activation of caspase 3 family members Blood, December 15, 2000; 96(13): 4204 - 4211. [Abstract] [Full Text] [PDF] I. Garcia-Higuera, Y. Kuang, J. Denham, and A. D. D'Andrea The Fanconi anemia proteins FANCA and FANCG stabilize each other and promote the nuclear accumulation of the Fanconi anemia complex Blood, November 1, 2000; 96(9): 3224 - 3230. [Abstract] [Full Text] [PDF] Y. Kuang, I. Garcia-Higuera, A. Moran, M. Mondoux, M. Digweed, and A. D. D'Andrea Carboxy terminal region of the Fanconi anemia protein, FANCG/XRCC9, is required for functional activity Blood, September 1, 2000; 96(5): 1625 - 1632. [Abstract] [Full Text] [PDF] N. C. Cheng, H. J. van de Vrugt, M. A. van der Valk, A. B. Oostra, P. Krimpenfort, Y. de Vries, H. Joenje, A. Berns, and F. Arwert Mice with a targeted disruption of the Fanconi anemia homolog Fanca Hum. Mol. Genet., July 22, 2000; 9(12): 1805 - 1811. [Abstract] [Full Text] [PDF] M. L. G. Dronkert, J. de Wit, M. Boeve, M. L. Vasconcelos, H. van Steeg, T. L. R. Tan, J. H. J. Hoeijmakers, and R. Kanaar Disruption of Mouse SNM1 Causes Increased Sensitivity to the DNA Interstrand Cross-Linking Agent Mitomycin C Mol. Cell. 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Mortimer, J. Mulcahy, R. K. Rathbun, G. Bagby, W. H. Fleming, and M. Grompe In Vivo Selection of Wild-Type Hematopoietic Stem Cells in a Murine Model of Fanconi Anemia Blood, September 15, 1999; 94(6): 2151 - 2158. [Abstract] [Full Text] [PDF] R. Abu-Issa, G. Eichele, and H. Youssoufian Expression of the Fanconi Anemia Group A Gene (Fanca) During Mouse Embryogenesis Blood, July 15, 1999; 94(2): 818 - 824. [Abstract] [Full Text] [PDF] I. Garcia-Higuera, Y. Kuang, D. Naf, J. Wasik, and A. D. D'Andrea Fanconi Anemia Proteins FANCA, FANCC, and FANCG/XRCC9 Interact in a Functional Nuclear Complex Mol. Cell. Biol., July 1, 1999; 19(7): 4866 - 4873. [Abstract] [Full Text] [PDF] L. S. Haneline, T. A. Gobbett, R. Ramani, M. Carreau, M. Buchwald, M. C. Yoder, and D. W. Clapp Loss of FancC Function Results in Decreased Hematopoietic Stem Cell Repopulating Ability Blood, July 1, 1999; 94(1): 1 - 8. [Abstract] [Full Text] [PDF] T. Yamashita, G. M. Kupfer, D. Naf, A. Suliman, H. Joenje, S. 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[Abstract] [Full Text] [PDF] M. Kanzaki and P. L. Morris Identification and Regulation of Testicular Interferon-{gamma} (IFN{gamma}) Receptor Subunits: IFN{gamma} Enhances Interferon Regulatory Factor-1 and Interleukin-1{beta} Converting Enzyme Expression Endocrinology, May 1, 1998; 139(5): 2636 - 2644. [Abstract] [Full Text] [PDF] M. Carreau, O. I. Gan, L. Liu, M. Doedens, C. McKerlie, J. E. Dick, and M. Buchwald Bone Marrow Failure in the Fanconi Anemia Group C Mouse Model After DNA Damage Blood, April 15, 1998; 91(8): 2737 - 2744. [Abstract] [Full Text] [PDF] M. E. Hoatlin, T. A. Christianson, W. W. Keeble, A. T. Hammond, Y. Zhi, M. C. Heinrich, P. A. Tower, and G. C. Bagby Jr The Fanconi Anemia Group C Gene Product Is Located in Both the Nucleus and Cytoplasm of Human Cells Blood, February 15, 1998; 91(4): 1418 - 1425. [Abstract] [Full Text] [PDF] M. C. Heinrich, M. E. Hoatlin, A. J. Zigler, K. V. Silvey, A. C. Bakke, W. W. Keeble, Y. Zhi, C. A. Reifsteck, M. Grompe, M. G. 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[Abstract] [Full Text] [PDF] G. M. Kupfer, T. Yamashita, D. Naf, A. Suliman, S. Asano, and A. D. D'Andrea The Fanconi Anemia Polypeptide, FAC, Binds to the Cyclin-Dependent Kinase, cdc2 Blood, August 1, 1997; 90(3): 1047 - 1054. [Abstract] [Full Text] [PDF] G. Pagano, L. G. Korkina, P. Degan, D. Del Principe, B. Lindau-Shepard, A. Zatterale, and C. Franceschi In Vitro Hypersensitivity to Oxygen of Fanconi Anemia (FA) Cells Is Linked to Ex Vivo Evidence for Oxidative Stress in FA Homozygotes and Heterozygotes Blood, February 1, 1997; 89(3): 1111 - 1111. [Full Text] [PDF] F. Qiao, A. Moss, and G. M. Kupfer Fanconi Anemia Proteins Localize to Chromatin and the Nuclear Matrix in a DNA Damage- and Cell Cycle-regulated Manner J. Biol. Chem., June 22, 2001; 276(26): 23391 - 23396. [Abstract] [Full Text] [PDF]

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