The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Pleiotropic defects in ataxia-telangiectasia protein-deficient mice
Open accessAbstract
We have generated a mouse model for ataxia-telangiectasia by using gene targeting to generate mice that do not express the Atm protein. Atm-deficient mice are retarded in growth, do not produce mature sperm, and exhibit severe defects in T cell maturation while going on to develop thymomas. Atm-deficient fibroblasts grow poorly in culture and display a high level of double-stranded chromosome breaks. Atm-deficient thymocytes undergo spontaneous apoptosis in vitro significantly more than controls. Atm-deficient mice then exhibit many of the same symptoms found in ataxia-telangiectasia patients and in cells derived from them. Furthermore, we demonstrate that the Atm protein exists as two discrete molecular species, and that loss of one or of both of these can lead to the development of the disease.
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Citations
Cites
A Single Ataxia Telangiectasia Gene with a Product Similar to PI-3 Kinase
Savitsky, K., Bar-Shira, A., Gilad, S., Rotman, G., Ziv, Y., Vanagaite, L., Tagle, D. A., Smith, S., Uziel, T., Sfez, S., et al. (1995) · Science · doi:10.1126/science.7792600 · Cited by 2,785
Cited by
The DNA damage response: putting checkpoints in perspective
Zhou, B.-B. S., & Elledge, S. J. (2000) · Nature · doi:10.1038/35044005 · Cited by 3,213
The ATM protein kinase: regulating the cellular response to genotoxic stress, and more
Shiloh, Y., & Ziv, Y. (2013) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3546 · Cited by 1,649
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
Chromosomal stability and the DNA double-stranded break connection
van Gent, D. C., Hoeijmakers, J. H. J., & Kanaar, R. (2001) · Nature Reviews Genetics · doi:10.1038/35056049 · Cited by 1,219
Regulation of oxidative stress by ATM is required for self-renewal of haematopoietic stem cells
Ito, K., Hirao, A., Arai, F., Matsuoka, S., Takubo, K., Hamaguchi, I., Nomiyama, K., Hosokawa, K., Sakurada, K., Nakagata, N., et al. (2004) · Nature · doi:10.1038/nature02989 · Cited by 1,175
Metadata
- Authors
- Elson, A., Wang, Y., Daugherty, C. J., Morton, C. C., Zhou, F., Campos-Torres, J., & Leder, P.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 1996-11-12
- Volume
- 93
- Issue
- 23
- Pages
- 13084-13089
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2033200608
- Cited by
- 496
- References
- 1
- FWCI
- 7.92
- Type
- journal article
- Language
- en
- Open access
- green
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- CoChat. (2026). Citation check: Pleiotropic defects in ataxia-telangiectasia protein-deficient mice. https://app.cochat.ai/papers/10.1073/pnas.93.23.13084
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- Elson, A., Wang, Y., Daugherty, C. J., Morton, C. C., Zhou, F., Campos-Torres, J., & Leder, P. (1996). Pleiotropic defects in ataxia-telangiectasia protein-deficient mice. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.93.23.13084
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