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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The MRN complex senses and processes double-strand DNA breaks and activates downstream DNA damage response proteins such as ATM.
“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]
Full activation of ATM after DNA double-strand breaks requires Nbs1 of the MRN complex.
“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]
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- 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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