mortality/aging
• few pups are recovered at E13.5
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Allele Symbol Allele Name Allele ID |
Tg(CMV-cre)1Ipc transgene insertion 1, I Pierre Chambon MGI:2177165 |
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Summary |
3 genotypes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• few pups are recovered at E13.5
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• severe dwarfism
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• reduced width of skull
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• narrow trunk
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• osteoclasts per bone area are increased
• osteoclast surface area/bone surface area is increased
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• fewer subchondral trabeculae
• trabeculae only partially mineralized
• more abundant collagen deposition
• significantly lower bone volume/trabecular volume in distal metaphysis of the femur
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• smaller hypertrophic mineralization zone
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• reduced size of epiphysis
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• defect in mineralization of calcified cartilage and primary spongiosa
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• reduced width of skull
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• osteoclasts per bone area are increased
• osteoclast surface area/bone surface area is increased
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• osteoclasts per bone area are increased
• osteoclast surface area/bone surface area is increased
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• wet weights of subcutaneous, infrarenal and intraperitoneal white adipose tissues are significantly increased by 30 weeks of age
• serum lipid parameters and food intake remain unaffected
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• inguinal testes
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• small degenerating testes
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• after 10 weeks of age, the growth of males increased dramatically
• by 12 weeks of age, the body weights of mutant males are higher than those of wild-type males
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• growth retardation in males through 10 weeks of age
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• clitoris-like phallus instead of a penis and scrotum
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• inguinal testes
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• small degenerating testes
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• clitoris-like phallus instead of a penis and scrotum
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• vagina with a blind end
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• feminization of external appearance, including a blind vagina and a clitoris-like phallus, with absence of internal male and female reproductive organs, except for atrophic testes
• no ovaries or uteri are observed
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• lower levels of androgens
• estradiol levels typical for a male
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• in response to angiotensin-II stimulation, male homozygotes show a smaller increase in the cross-sectional area of cardiomyocytes relative to wild-type males
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• after 6 weeks of age, male homozygotes show a significantly reduced cross-sectional area of cardiomyocytes in LV tissues relative to wild-type males
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• after 6 weeks of age, male homozygotes exhibit a cardiac size reduction relative to wild-type males
• in response to angiotensin-II stimulation, male homozygotes show a smaller increase in cardiac size relative to wild-type males
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• male homozygotes display no significant differences in basal levels of systolic blood pressure and heart rate; however, after 6 weeks of age, male homozygotes show a significant reduction in HW/BW ratio relative to wild-type males
• in response to angiotensin-II stimulation, male homozygotes display a smaller increase in HW/BW ratio relative to wild-type males
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• after 6 weeks of age, male homozygotes display a reduced LV volume relative to wild-type males
• in response to angiotensin-II stimulation, male homozygotes exhibit an impaired hypertrophic response with lower cardiac LV volumes that is partly associated with reduced activation of mitogen-activated protein kinases 1/2 and 5
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• after 6 weeks of age, male homozygotes exhibit a reduced left ventricular (LV) wall thickness relative to wild-type males
• in response to angiotensin-II stimulation, male homozygotes show a lower increase in LV wall thickening relative to wild-type males
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• in response to angiotensin-II stimulation, 25-wk-old male homozygotes display an exacerbated interstitial fibrosis in the left ventricular area relative to age-matched wild-type males
• enhanced angiotensin-II-mediated cardiac interstitial fibrosis is associated with up-regulation of collagen I and III gene expression and enhanced cardiac transforming growth factor-beta1 and Smad2 activation
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• at 25 weeks, male homozygotes exhibit a normal left ventricular fractional shortening (a marker of systolic function) relative to wild-type males
• however, in response to angiotensin-II stimulation, male homozygotes display a significantly attenuated LV systolic function relative to wild-type males
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• in response to angiotensin-II stimulation, male homozygotes show a smaller increase in the cross-sectional area of cardiomyocytes relative to wild-type males
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• after 6 weeks of age, male homozygotes show a significantly reduced cross-sectional area of cardiomyocytes in LV tissues relative to wild-type males
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• at 25 weeks, male homozygotes exhibit a normal left ventricular fractional shortening (a marker of systolic function) relative to wild-type males
• however, in response to angiotensin-II stimulation, male homozygotes display a significantly attenuated LV systolic function relative to wild-type males
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• in response to angiotensin-II stimulation, 25-wk-old male homozygotes display an exacerbated interstitial fibrosis in the left ventricular area relative to age-matched wild-type males
• enhanced angiotensin-II-mediated cardiac interstitial fibrosis is associated with up-regulation of collagen I and III gene expression and enhanced cardiac transforming growth factor-beta1 and Smad2 activation
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
androgen insensitivity syndrome | DOID:4674 |
OMIM:300068 |
J:85484 | |
obesity | DOID:9970 |
OMIM:601665 |
J:85484 |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/12/2024 MGI 6.24 |
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