A murine model of Nijmegen breakage syndrome

Current Biology

Volumn 12, Issue 8, 2002, Pages 648-653

  Williams, Bret R ^a,b   Mirzoeva, Olga K ^a   Morgan, William F ^c   Lin, Junyu ^a   Dunnick, Wesley ^d   Petrini, John H J ^a,b  

^a UNIVERSITY OF WISCONSIN SCHOOL OF MEDICINE AND PUBLIC HEALTH   (United States)

^b UNIVERSITY OF WISCONSIN MADISON   (United States)

^c UNIVERSITY OF MARYLAND SCHOOL OF MEDICINE   (United States)

^d UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ATM PROTEIN; CELL CYCLE PROTEIN; DNA BINDING PROTEIN; MESSENGER RNA; MRE11A PROTEIN, HUMAN; MRE11A PROTEIN, MOUSE; NIJMEGEN BREAKAGE SYNDROME 1 PROTEIN, MOUSE; NUCLEAR PROTEIN; POLYDEOXYRIBONUCLEOTIDE SYNTHASE; PROTEIN SERINE THREONINE KINASE; TUMOR SUPPRESSOR PROTEIN;

AMINO ACID SEQUENCE; ANIMAL; ARTICLE; CELL CYCLE; CHEMISTRY; CHROMOSOME BREAKAGE; DISEASE MODEL; DNA DAMAGE; FIBROBLAST; GENE DELETION; GENETIC EPISTASIS; GENETICS; HUMAN; LETHAL GENE; METABOLISM; MOLECULAR GENETICS; MOUSE; MOUSE MUTANT; MUTATION; PHENOTYPE; SOUTHERN BLOTTING; SYNDROME;

AMINO ACID SEQUENCE; ANIMALS; BLOTTING, SOUTHERN; CELL CYCLE; CELL CYCLE PROTEINS; CHROMOSOME BREAKAGE; DISEASE MODELS, ANIMAL; DNA DAMAGE; DNA REPAIR ENZYMES; DNA-BINDING PROTEINS; EPISTASIS, GENETIC; FIBROBLASTS; GENE DELETION; GENES, LETHAL; HUMANS; MICE; MICE, KNOCKOUT; MOLECULAR SEQUENCE DATA; MUTATION; NUCLEAR PROTEINS; PHENOTYPE; PROTEIN-SERINE-THREONINE KINASES; RNA, MESSENGER; SYNDROME; TUMOR SUPPRESSOR PROTEINS;

EID: 0037117410     PISSN: 09609822     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0960-9822(02)00763-7     Document Type: Article

References (28)

1. Nijmegen breakage syndrome
2. Nibrin, a novel DNA double-strand break repair protein, is mutated in Nijmegen breakage syndrome
3. The hMre11/hRad50 protein complex and Nijmegen breakage syndrome: Linkage of double-strand break repair to the cellular DNA damage response
4. The Mre11-Rad50 complex: Diverse functions in the cellular DNA damage response
5. The mre11 complex and ATM: Collaborating to navigate S phase
6. Ataxia-telangiectasia and the Nijmegen breakage syndrome: Related disorders but genes apart
7. ATM-dependent phosphorylation of nibrin in response to radiation exposure
8. Functional link between ataxia-telangiectasia and Nijmegen breakage syndrome gene products
9. ATM phosphorylation of Nijmegen breakage syndrome protein is required in a DNA damage response
10. ATM phosphorylates p95/Nbs1 in an S-phase checkpoint pathway
11. An alternative mode of translation permits production of a variant NBS1 protein from the common Nijmegen breakage syndrome allele
12. Identification, characterization, and mapping of a mouse homolog of the gene mutated in Nijmegen breakage syndrome
13. Targeted disruption of the Nijmegen breakage syndrome gene NBS1 leads to early embryonic lethality in mice
14. Distinct functional domains of nibrin mediate Mre11 binding, focus formation, and nuclear localization
15. Nijmegen breakage syndrome cells fail to induce the p53-mediated DNA damage response following exposure to ionizing radiation
16. Radiation induction of p53 in cells from Nijmegen breakage syndrome is defective but not similar to ataxia-telangiectasia
17. Characterization of cell cycle checkpoint responses after ionizing radiation in Nijmegen breakage syndrome cells
18. Cell cycle checkpoints and DNA repair in Nijmegen breakage syndrome
19. The DNA double strand break repair gene hMre11 is mutated in individuals with a new ataxia telangiectasia like disorder (ATLD)
20. Involvement of Brca1 in S-phase and G(2)-phase checkpoints after ionizing irradiation
21. Chk2 activation dependence on Nbs1 after DNA damage
22. Tumor spectrum analysis in p53-mutant mice
23. Atm-deficient mice: A paradigm of ataxia telangiectasia
24. The MRE11 complex and DNA replication: Linkage to E2F and sites of DNA synthesis
25. Overexpression of a kinase-inactive ATR protein causes sensitivity to DNA-damaging agents and defects in cell cycle checkpoints
26. ATR/ATM-mediated phosphorylation of human Rad17 is required for genotoxic stress responses
27. Functional interactions between BRCA1 and the checkpoint kinase ATR during genotoxic stress
28. ATR and ATRIP: Partners in checkpoint signaling