mortality/aging
• a sharp decline in survival is noted after P15
• none of the mice survive beyond 26 days of life
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growth/size/body
• at 2 wks of age, mice are readily identified by their smaller body size and sickly appearance
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renal/urinary system
• neonatal kidneys display kinked collecting ducts
• DBA staining of collecting tubules within the cortex indicates higher density and cystic morphology
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• at P14, the density of glomeruli within the outermost cortical layer is significantly increased
• at P14, most glomeruli are small and unevenly positioned within the cortex (i.e. subcapsular)
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• at P14, aberrant subcapsular glomeruli show little evidence of capillary blood flow
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• in vitro, E11.5 kidneys cultured for 48 hrs in serum free media display subtle branching defects including asymmetric ureteric bud tips that are often dilated, abnormally enlarged misshapen ampullae, discontinuous and jagged laminin basement membranes, kinked ureteric bud stalks and small ectopic bud outgrowths
• neonatal kidneys exhibit terminal end buds that are often dilated and connected to misshaped stalks
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• in vitro, E11.5 kidneys cultured for 48 hrs in serum free media display small ectopic bud outgrowths
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cardiovascular system
• at P14, aberrant subcapsular glomeruli show little evidence of capillary blood flow
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