mortality/aging
• all mice are born healthy but die of renal failure within 4 to 5 weeks of birth
• however, mice appear normal up to 2 weeks after birth
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growth/size/body
• at ~4 weeks of age, mice exhibit significant growth retardation relative to controls
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renal/urinary system
• at ~4 weeks of age, mice exhibit significant proteinuria relative to controls
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• at 4 weeks, nephrin expression appears reduced while the polarity protein Par3, the tight junction marker ZO-1, and the slit diaphragm molecules nephrin and podocin exhibit a significantly impaired, granular distribution along the glomerular basement membrane, unlike the linear pattern seen in wild-type controls
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• at ~4 weeks of age, foot processes appear globally effaced
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• at ~4 weeks of age, severe junctional abnormalities are observed
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• at ~4 weeks of age, foot processes often display significant slit diaphragm displacement
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• at ~4 weeks of age, mice develop segmental glomerulosclerosis
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• at ~4 weeks of age, mice exhibit renal tubule dilation
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renal cast
(
J:164628
)
• at ~4 weeks of age, mice exhibit proteinuric casts in the tubule system
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• mice develop nephrotic syndrome and die of renal failure
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homeostasis/metabolism
• at ~4 weeks of age, mice exhibit significant proteinuria relative to controls
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