| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
|
|
|
|||||||
|
|||||||||
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Papers In Press, published online ahead of print October 1, 2002
Pharmacology, University of Minnesota, Minneapolis, MN 55455
Corresponding Author: dona0054{at}tc.umn.edu
Fanconi anemia (FA) is a heterogeneous autosomal recessive disease characterized by congenital abnormalities, pancytopenia, and an increased incidence of cancer. Cells cultured from FA patients display elevated spontaneous chromosomal breaks and deletions, and are hypersensitive to bifunctional crosslinking agents. Thus it has been hypothesized that FA is a DNA repair disorder. We analyzed plasmid end-joining in intact diploid fibroblast cells derived from FA patients. FA fibroblasts from complementation groups A, C, D2, and G rejoined linearized plasmids with a significantly decreased efficiency compared to non-FA fibroblasts. Retrovirus-mediated expression of the respective FA cDNAs in FA cells restored their end-joining efficiency to wild type levels. Human FA fibroblasts and fibroblasts from FA rodent models were also significantly more sensitive to restriction enzyme-induced chromosomal DNA double-strand breaks than were their retrovirally-corrected counterparts. Taken together these data show that FA fibroblasts have a deficiency in both extrachromosomal and chromosomal DNA double-strand break repair, a defect that could provide an attractive explanation for some of the pathologies associated with FA.
J. Biol. Chem, 10.1074/jbc.M207937200
Submitted on August 5, 2002
Revised on September 27, 2002
Accepted on September 27, 2002
A DNA double-strand break repair defect in fanconi anemia fibroblasts
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  J. M. Hinz, P. B. Nham, S. S. Urbin, I. M. Jones, and L. H. Thompson Disparate contributions of the Fanconi anemia pathway and homologous recombination in preventing spontaneous mutagenesis Nucleic Acids Res., June 28, 2007; 35(11): 3733 - 3740. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Sobeck, S. Stone, and M. E. Hoatlin DNA Structure-Induced Recruitment and Activation of the Fanconi Anemia Pathway Protein FANCD2 Mol. Cell. Biol., June 15, 2007; 27(12): 4283 - 4292. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Sobeck, S. Stone, V. Costanzo, B. de Graaf, T. Reuter, J. de Winter, M. Wallisch, Y. Akkari, S. Olson, W. Wang, et al. Fanconi Anemia Proteins Are Required To Prevent Accumulation of Replication-Associated DNA Double-Strand Breaks Mol. Cell. Biol., January 15, 2006; 26(2): 425 - 437. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Houghtaling, A. Newell, Y. Akkari, T. Taniguchi, S. Olson, and M. Grompe Fancd2 functions in a double strand break repair pathway that is distinct from non-homologous end joining Hum. Mol. Genet., October 15, 2005; 14(20): 3027 - 3033. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y.-G. Yang, Z. Herceg, K. Nakanishi, I. Demuth, C. Piccoli, J. Michelon, G. Hildebrand, M. Jasin, M. Digweed, and Z.-Q. Wang The Fanconi anemia group A protein modulates homologous repair of DNA double-strand breaks in mammalian cells Carcinogenesis, October 1, 2005; 26(10): 1731 - 1740. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W.-H. Park, S. Margossian, A. A. Horwitz, A. M. Simons, A. D. D'Andrea, and J. D. Parvin Direct DNA Binding Activity of the Fanconi Anemia D2 Protein J. Biol. Chem., June 24, 2005; 280(25): 23593 - 23598. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. Nakanishi, Y.-G. Yang, A. J. Pierce, T. Taniguchi, M. Digweed, A. D. D'Andrea, Z.-Q. Wang, and M. Jasin Human Fanconi anemia monoubiquitination pathway promotes homologous DNA repair PNAS, January 25, 2005; 102(4): 1110 - 1115. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. E. Lamerdin, N. A. Yamada, J. W. George, B. Souza, A. T. Christian, N. J. Jones, and L. H. Thompson Characterization of the hamster FancG/Xrcc9 gene and mutations in CHO UV40 and NM3 Mutagenesis, May 1, 2004; 19(3): 237 - 244. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. L. Donahue, R. Lundberg, R. Saplis, and C. Campbell Deficient Regulation of DNA Double-strand Break Repair in Fanconi Anemia Fibroblasts J. Biol. Chem., August 8, 2003; 278(32): 29487 - 29495. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Tonnies, S. Huber, J.-S. Kuhl, A. Gerlach, W. Ebell, and H. Neitzel Clonal chromosomal aberrations in bone marrow cells of Fanconi anemia patients: gains of the chromosomal segment 3q26q29 as an adverse risk factor Blood, May 15, 2003; 101(10): 3872 - 3874. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
| All ASBMB Journals | Molecular and Cellular Proteomics |
| Journal of Lipid Research | ASBMB Today |
