mortality/aging
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• males die within 1 week of birth
• survival of females is similar to controls
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renal/urinary system
• thin bladder wall
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• thin smooth muscle layer
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• grossly dilated bladders
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• renal urothelium of severely affected kidneys contains numerous multilayered regions of 3-5 cells throughout the renal pelvis and show frequent cellular blebbing compared to controls that have a single layer of cuboidal cells with intermittent multilayered regions of 2-3 cells
• severely affected kidneys show a 20-fold increase in the percentage of cellular proliferation within the renal urothelium
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muscle
• thin smooth muscle layer
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