The DOI matches its Crossref record; 47 of the 47 references it cites were found in a registry or an index.
Role of Nbs1 in the activation of the Atm kinase revealed in humanized mouse models
Abstract
Nijmegen breakage syndrome (NBS) is a chromosomal fragility disorder that shares clinical and cellular features with ataxia telangiectasia. Here we demonstrate that Nbs1-null B cells are defective in the activation of ataxia-telangiectasia-mutated (Atm) in response to ionizing radiation, whereas ataxia-telangiectasia- and Rad3-related (Atr)-dependent signalling and Atm activation in response to ultraviolet light, inhibitors of DNA replication, or hypotonic stress are intact. Expression of the main human NBS allele rescues the lethality of Nbs1-/- mice, but leads to immunodeficiency, cancer predisposition, a defect in meiotic progression in females and cell-cycle checkpoint defects that are associated with a partial reduction in Atm activity. The Mre11 interaction domain of Nbs1 is essential for viability, whereas the Forkhead-associated (FHA) domain is required for T-cell and oocyte development and efficient DNA damage signalling. Reconstitution of Nbs1 knockout mice with various mutant isoforms demonstrates the biological impact of impaired Nbs1 function at the cellular and organismal level.
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In the citing paper
“The MRN complex is important in sensing and processing double-strand DNA breaks, as well as activating downstream DNA damage response proteins that mediate or signal for DNA repair, such as the DNA damage-induced kinase ATM.9–13”
From Mdmx promotes genomic instability independent of p53 and Mdm2 (2015) · cites this paper as [13]
In the citing paper
“Full activation of ATM following DNA double-strand breaks requires Nbs1 of the MRN complex.9,11–13”
From Mdmx promotes genomic instability independent of p53 and Mdm2 (2015) · cites this paper as [13]
Citations
Cites
DNA damage activates ATM through intermolecular autophosphorylation and dimer dissociation
Bakkenist, C. J., & Kastan, M. B. (2003) · Nature · doi:10.1038/nature01368 · Cited by 3,466
ATM Activation by DNA Double-Strand Breaks Through the Mre11-Rad50-Nbs1 Complex
Lee, J.-H., & Paull, T. T. (2005) · Science · doi:10.1126/science.1108297 · Cited by 1,424
Genomic Instability in Mice Lacking Histone H2AX
Celeste, A., Petersen, S., Romanienko, P. J., Fernandez-Capetillo, O., Chen, H. T., Sedelnikova, O. A., Reina-San-Martin, B., Coppola, V., Meffre, E., Difilippantonio, M. J., et al. (2002) · Science · doi:10.1126/science.1069398 · Cited by 1,385
Self-citationConserved modes of recruitment of ATM, ATR and DNA-PKcs to sites of DNA damage
Falck, J., Coates, J., & Jackson, S. P. (2005) · Nature · doi:10.1038/nature03442 · Cited by 1,304
Requirement of the MRN complex for ATM activation by DNA damage
Uziel, T., Lerenthal, Y., Moyal, L., Andegeko, Y., Mittelman, L., & Shiloh, Y. (2003) · The EMBO Journal · doi:10.1093/emboj/cdg541 · Cited by 1,075
Histone H2AX phosphorylation is dispensable for the initial recognition of DNA breaks
Celeste, A., Fernandez-Capetillo, O., Kruhlak, M. J., Pilch, D. R., Staudt, D. W., Lee, A., Bonner, R. F., Bonner, W. M., & Nussenzweig, A. (2003) · Nature Cell Biology · doi:10.1038/ncb1004 · Cited by 989
Self-citationThe MRE11 complex: at the crossroads of DNA repair and checkpoint signalling
D'Amours, D., & Jackson, S. P. (2002) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm805 · Cited by 874
ATM phosphorylates p95/nbs1 in an S-phase checkpoint pathway
Lim, D.-S., Kim, S.-T., Xu, B., Maser, R. S., Lin, J., Petrini, J. H. J., & Kastan, M. B. (2000) · Nature · doi:10.1038/35007091 · Cited by 786
Direct Activation of the ATM Protein Kinase by the Mre11/Rad50/Nbs1 Complex
Lee, J.-H., & Paull, T. T. (2004) · Science · doi:10.1126/science.1091496 · Cited by 762
H2AX Haploinsufficiency Modifies Genomic Stability and Tumor Susceptibility
Celeste, A., Difilippantonio, S., Difilippantonio, M. J., Fernández-Capetillo, Ó., Pilch, D. R., Sedelnikova, O. A., Eckhaus, M., Ried, T., Bonner, W. M., & Nussenzweig, A. (2003) · Cell · doi:10.1016/s0092-8674(03)00567-1 · Cited by 558
Self-citation
Cited by
Sorted by citations
Mechanism and Regulation of Class Switch Recombination
Stavnezer, J., Guikema, J. E. J., & Schrader, C. E. (2008) · Annual Review of Immunology · doi:10.1146/annurev.immunol.26.021607.090248 · Cited by 1,195
The MRE11 complex: starting from the ends
Stracker, T. H., & Petrini, J. H. J. (2011) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3047 · Cited by 734
Lou, Z., Minter‐Dykhouse, K., Franco, S., Gostissa, M., Rivera, M. A., Celeste, A., Manis, J. P., van Deursen, J., Nussenzweig, A., Paull, T. T., et al. (2006) · Molecular Cell · doi:10.1016/j.molcel.2005.11.025 · Cited by 668
Self-citationActivation and regulation of ATM kinase activity in response to DNA double-strand breaks
Lee, J.-H., & Paull, T. T. (2007) · Oncogene · doi:10.1038/sj.onc.1210872 · Cited by 545
The ATM-mediated DNA-damage response: taking shape
Shiloh, Y. (2006) · Trends in Biochemical Sciences · doi:10.1016/j.tibs.2006.05.004 · Cited by 556
Metadata
- Authors
- Difilippantonio, S., Celeste, A., Fernandez-Capetillo, O., Chen, H.-T., Martin, B. R. S., Laethem, F. V., Yang, Y.-P., Petukhova, G. V., Eckhaus, M., Feigenbaum, L., Manova, K., Kruhlak, M., Camerini-Otero, R. D., Sharan, S., Nussenzweig, M., & Nussenzweig, A.
- Affiliations
- National Institutes of Health (US)
- National Cancer Institute (US)
- Memorial Sloan Kettering Cancer Center (US)
- Howard Hughes Medical Institute (US)
- Corresponding author
- Nussenzweig, André
- ORCID
- Venue
- Nature Cell Biology
- Published
- 2005-06-19
- Volume
- 7
- Issue
- 7
- Pages
- 675-685
- ISSN
- 1465-7392 (ISSN-L)
- 1476-4679
- OpenAlex
- W2152456839
- Cited by
- 227
- References
- 47
- Citations by year
- 2025: 2 · 2024: 2 · 2023: 4 · 2022: 1 · 2021: 2 · 2020: 4 · 2019: 7 · 2018: 4 · 2017: 4 · 2016: 2 · 2015: 7 · 2014: 12 · 2013: 16 · 2012: 15
- FWCI
- 12.17
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Cell Biology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Effects of Radiation Exposure
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
- Keywords
- NBS1, ATM kinase activation, Nijmegen breakage syndrome, ataxia telangiectasia, ATR kinase, ionizing radiation response, DNA replication inhibitors, hypotonic stress, forkhead-associated domain, DNA damage signaling, meiotic progression, cell cycle checkpoints, immunodeficiency, cancer susceptibility, humanized mice
- MeSH terms
- MRE11 Homologue Protein, Animals, B-Lymphocytes, Binding Sites, Cell Cycle, Chromosome Aberrations, Disease Models, Animal, DNA Damage, DNA Replication, DNA-Binding Proteins, Female, Gonads, Humans, Lymphoma, Non-Hodgkin, Male, Mice, Transgenic, Mutation, Nuclear Proteins, Phosphorylation, Syndrome, T-Lymphocytes, Tumor Suppressor Protein p53, Protein Serine-Threonine Kinases, Immunoglobulin Class Switching, Acid Anhydride Hydrolases, Mice, Knockout, ATP-Binding Cassette Transporters, Cell Cycle Proteins, Chromosome Breakage, Genetic Predisposition to Disease, Chromosome Disorders, Tumor Suppressor Proteins, DNA Repair Enzymes, Mice, Ataxia Telangiectasia Mutated Proteins
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