mortality/aging
• average lifespan is 4.6 months
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renal/urinary system
• Background Sensitivity: at 2 to 8 months, 6 of 9 male mice from the first generation (N1) on a background containing FVB exhibit heavy proteinuria unlike mice backcrossed to a C57BL/6 background for 4 generations
• all mice from N2, N3, and N4 generations on the FVB containing background develop proteinuria by 2 weeks and 2 months unlike wild-type mice
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• parietal cells stain for anti-IgG and anti-IgM antibodies unlike in wild-type mice
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• in male mice, GBM thickening is sometimes associated with podocyte hypertrophy and vacuolation unlike in wild-type mice
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• in male mice, GBM thickening is sometimes associated with podocyte hypertrophy
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• by the onset of proteinuria, male mice develop a GBM thickening that is sometimes associated with podocyte hypertrophy and vacuolation unlike in wild-type mice
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• glomerular lesions are frequently more severe at the periphery of the cortex with enlarged deeper glomeruli that are less affected
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• mesangial cells stain for anti-IgG and anti-IgM antibodies unlike in wild-type mice
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• mice develop mesangial slerosis unlike in wild-type mice
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• mice develop mesangiolysis unlike in wild-type mice
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• all mice develop glomerulosclerosis with tubular damages with female mice developing renal pathologies later than male mice
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homeostasis/metabolism
• Background Sensitivity: at 2 to 8 months, 6 of 9 male mice from the first generation (N1) on a background containing FVB exhibit heavy proteinuria unlike mice backcrossed to a C57BL/6 background for 4 generations
• all mice from N2, N3, and N4 generations on the FVB containing background develop proteinuria by 2 weeks and 2 months unlike wild-type mice
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cellular
• mesangial cells stain for anti-IgG and anti-IgM antibodies unlike in wild-type mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Denys-Drash syndrome | DOID:3764 |
OMIM:194080 |
J:154995 |