Analysis Tools
mortality/aging
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• fail to thrive and die around P24
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cellular
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• increased sensitivity of MEFs to monofunctional alkylating agents suggesting a defect in base excision repair; however double strand break repair and nucleotide excision repair are similar to wild-type
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• increased DNA damage and reduced survival in MEFs and ES cells following exposure to ionizing radiation; however, UV sensitivity of MEFs is similar to wild-type cells
• thymocytes from 21 day old mice display increased sensitivity to IR and monofunctional alkylating agents; however sensitivity of cells from 12 day old mice is similar to wild-type
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• in MEFs and ES cells
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• increase in the frequency of fragmented chromosome, detached centromeres and chromosomal gaps in metaphase spreads from early and late passage MEFs
• spectral karyotype analysis detects increases in the frequency of chromosome breaks, translocations, and dicentric chromosomes
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growth/size/body
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• significantly smaller by about 10 days of age
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• decreased body weight as compared to controls at 3 weeks of age on standard diet
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• decreased body length as compared to controls at 3 weeks of age on standard diet
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• similar size at birth but significantly smaller by about 10 days of age; however food intake is normal
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adipose tissue
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• acute loss beginning around 3 weeks of age
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digestive/alimentary system
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• consisting of erosion of the superficial colonic epithelium beginning around 3 weeks of age
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hematopoietic system
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• 10-fold reduction in the number of splenic lymphoctes
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• 10-fold reduction in the number of B cell progenitors in the bone marrow
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• 50-fold reduction in the thymus
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• 10-fold reduction in the number of B cell progenitors in the bone marrow
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• severe lymphopenia associated with increased lymphocyte apoptosis
• adoptive transfer studies indicate increased lymphocyte apoptosis is not a cell intrinsic defect
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• 10-fold reduction in the number of splenic lymphocytes
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homeostasis/metabolism
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• decreases rapidly in mice older than 12 days of age, dropping below detection limits by 24 days of age
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• serum levels of insulin like growth factor (IGF1) are low at 12 days of age and severely by 19 days of age
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skeleton
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• lordokyphosis visible around 3 weeks of age
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• 30% reduction in bone mineral density at about 3 weeks of age
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immune system
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• consisting of erosion of the superficial colonic epithelium beginning around 3 weeks of age
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• 10-fold reduction in the number of splenic lymphoctes
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• 10-fold reduction in the number of B cell progenitors in the bone marrow
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• 50-fold reduction in the thymus
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• severe lymphopenia associated with increased lymphocyte apoptosis
• adoptive transfer studies indicate increased lymphocyte apoptosis is not a cell intrinsic defect
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• 10-fold reduction in the number of splenic lymphocytes
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integument
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• acute loss beginning around 3 weeks of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| progeria | DOID:3911 |
OMIM:176670 |
J:112817 | |
