Analysis Tools
mortality/aging
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• slightly fewer (20.6%) than the expected (25%) numbers of mice are obtained, indicating a low rate of embryonic lethality or reduced postnatal viability
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behavior/neurological
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• mice exhibit an overall increase in the foot spacing consistent with a wide-spaced ataxic gait
• mice show a decrease in the stance time of all 4 limbs of locomotion and a trend toward increased front and rear paw swing time at 3 months of age
• however, mice exhibit normal performance on the rotarod at 4, 7, and 9 months of age
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cellular
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• cerebellar granule neurons show decreased susceptibility to radiomimetic-induced cell death, with more surviving cerebellar granule cells in cultures 20 hours after addition of doxorubicin
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• fibroblasts grown in culture proliferate at a reduced rate compared to controls
• however, oxidative stress is not seen
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• fibroblasts exhibit an altered DNA damage response
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endocrine/exocrine glands
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• mice rarely develop thymic lymphomas or other cancers, with 8% of mice succumbing to thymic lymphomas
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hematopoietic system
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• increase in the percentage of B cells within the circulating lymphocyte populations at 2 and 5 months of age
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• reduction in the percentage of T-cell subsets within the circulating lymphocyte population at 2 and 5 months of age
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homeostasis/metabolism
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• fibroblasts exhibit an altered DNA damage response
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immune system
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• increase in the percentage of B cells within the circulating lymphocyte populations at 2 and 5 months of age
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• reduction in the percentage of T-cell subsets within the circulating lymphocyte population at 2 and 5 months of age
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neoplasm
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• mice rarely develop thymic lymphomas or other cancers, with 8% of mice succumbing to thymic lymphomas
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nervous system
| N |
• no cellular defects are seen in the cerebellum
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| ataxia telangiectasia | DOID:12704 |
OMIM:208900 |
J:226414 | |
