Analysis Tools
mortality/aging
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• animals with thymic lymphomas die by 4 months of age due to size of tumor that disrupts the thoracic cavity
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growth/size/body
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• homozygous mice weigh approximately 88% of controls at 3 weeks of age; the weight is 75% of controls at 5 weeks of age and persists through adulthood
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cellular
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• 72 hours after serum stimulation, starved MEFs have reduced numbers of cells in S phase compared to controls, indicating a defect in cell cycle progression
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• meiosis is disrupted prior to normal meiotic arrest in females
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• development appears to arrest between the zygotene and pachytene stages of meiotic prophase
• spermatocytes are found in various stages of degeneration and round or elongated spermatids are absent
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• homozygous ES cells have a reduced survival rate compared to controls when exposed to increasing dosages of gamma-irradiation
• homozygous MEFs exhibited defective cell cycle arrest rates and decreased up-regulation of Trp53 following gamma-irradiation, and exhibited a
• mutant thymocytes are more resistant to gamma-irradiation induced apoptosis than control cells
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• homozygous MEFs grow slowly in culture and achieve senesence by passage 6
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neoplasm
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• tumors form in almost all mice by 3 months of age, and are often seen in 1-2 month old animals
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reproductive system
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• meiosis is disrupted prior to normal meiotic arrest in females
|
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• development appears to arrest between the zygotene and pachytene stages of meiotic prophase
• spermatocytes are found in various stages of degeneration and round or elongated spermatids are absent
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• ovaries are degenerate and contain no primary oocytes or follicles
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immune system
small thymus
(
J:35914
)
|
• 60% decrease in the single positive and double positive thymocyte subpopulations
• particular reduction in the proportion of CD4+ single positive thymocytes
|
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• tumors form in almost all mice by 3 months of age, and are often seen in 1-2 month old animals
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• decreased number of B220+IgM- pre-B cells in the bone marrow (63% of controls)
• similar numbers of B220+CD43+ pre-B cells are present compared to controls
• similar numbers of B cells are seen in the spleen when compared to controls
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• a 1.5-2 fold decrease in serum levels of IgG2a, IgG2b and IgG3 is seen
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• T cell-dependent immune respose to NP15-CG is impaired in homozygous mice
• T cell-independent immune responses are similar to controls
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• widespread microglial activation associated with neuronal loss
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nervous system
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• widespread microglial activation associated with neuronal loss
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• 21% exhibit degenerating granule cells
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• 46.5% exhibit degenerating neurons in the molecular layer
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• although earlier studies using light microscopy did not reveal neuron degeneration, detailed electron microscopy reveals widespread neuronal degeneration and glial activation
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• 33% exhibit degenerating/dystrophic Purkinje cells
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behavior/neurological
| N |
• no ataxia or other behavioral abnormalities are seen
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endocrine/exocrine glands
small thymus
(
J:35914
)
|
• 60% decrease in the single positive and double positive thymocyte subpopulations
• particular reduction in the proportion of CD4+ single positive thymocytes
|
|
• tumors form in almost all mice by 3 months of age, and are often seen in 1-2 month old animals
|
|
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• ovaries are degenerate and contain no primary oocytes or follicles
|
hematopoietic system
small thymus
(
J:35914
)
|
• 60% decrease in the single positive and double positive thymocyte subpopulations
• particular reduction in the proportion of CD4+ single positive thymocytes
|
|
• tumors form in almost all mice by 3 months of age, and are often seen in 1-2 month old animals
|
|
• decreased number of B220+IgM- pre-B cells in the bone marrow (63% of controls)
• similar numbers of B220+CD43+ pre-B cells are present compared to controls
• similar numbers of B cells are seen in the spleen when compared to controls
|
|
• a 1.5-2 fold decrease in serum levels of IgG2a, IgG2b and IgG3 is seen
|
|
• T cell-dependent immune respose to NP15-CG is impaired in homozygous mice
• T cell-independent immune responses are similar to controls
|
|
• widespread microglial activation associated with neuronal loss
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| ataxia telangiectasia | DOID:12704 |
OMIM:208900 |
J:42324 , J:44190 | |
