Analysis Tools
mortality/aging
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• ~5% of mice failed to survive either birth or the first week of life due to complete bilateral renal agenesis
• all others survived into adulthood (8 weeks), indicating at least one functioning kidney
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renal/urinary system
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• a significant increase in apoptotic (TUNEL+) cells was observed in the cortex
• a significant increase in apoptotic (TUNEL+) cells was associated with the dilated tubules in the medulla
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• a significant increase in proliferative (Ki67+) cells was noted in the cortex, probably associated with proximal tubular structures, but not in the medulla
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• adult mice displayed an increased urinary AVP-to-creatinine ratio (as a surrogate for plasma AVP levels)
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• adult mice exhibited a severe decrease in urine osmolarity relative to control mice
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• mice exhibited a wide range of kidney abnormalities ranging from bilateral agenesis to grossly normal-sized kidneys
• mice with polyuria and renal failure showed a severely disturbed kidney architecture
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• adult renal medullary collecting ducts exhibited hypoplasia, increased apoptosis, dilation (ectasia) and cyst formation
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• adult renal medullary collecting ducts exhibited ectasia
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• in adult mice, normal renal cortex areas containing clustered glomeruli were separated by larger areas with multiple cystic structures, likely to be dilated cortical collecting ducts
• a significant increase in apoptotic (TUNEL+) cells was observed in the cortex
• a significant increase in proliferative (Ki67+) cells was noted in the cortex, probably associated with proximal tubular structures, but not in the medulla
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• in adult mice, the renal medulla exhibited tubular ectasia
• a significant increase in apoptotic (TUNEL+) cells was associated with the dilated tubules in the medulla
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• adult renal medullary collecting ducts exhibited cyst formation
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• late-stage developing kidneys displayed a reduced inner medulla size
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small kidney
(
J:149831
)
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• at both 1 and 2 wks of age, mutant kidneys were smaller than control kidneys
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• at both 1 and 2 weeks of age
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• at E18-P1, eleven of 25 mice showed gross renal hypoplasia, with mutant kidneys being less than 2/3 of normal size
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• 2 of 45 mice (~5%) displayed complete bilateral renal agenesis
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• at E18-P1, one of 25 mice displayed unilateral renal agenesis
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• adult kidneys exhibited a reduced capacity to clear the contrast agent DPTA from blood
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• in adult mice, renal uptake of labeled PAH was reduced ~85% relative to that of control kidneys
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isosthenuria
(
J:149831
)
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• mutant urine remained relatively isosmotic with blood plasma
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• in mice with polyuria and severely disturbed kidney architecture
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• at 8 weeks of age, mice had >10 times the urine output seen in control mice
• water deprivation for 12 hrs failed to reduce the urine output, unlike in control mice
• polyuria is likely due to an inability to respond to AVP within the kidney
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homeostasis/metabolism
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• adult mice displayed increased plasma urea levels
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• adult mice displayed an increased urinary AVP-to-creatinine ratio (as a surrogate for plasma AVP levels)
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• adult mice exhibited a severe decrease in urine osmolarity relative to control mice
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hematopoietic system
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• adult mice displayed a significantly lower blood hematocrit than control mice (42% in vs. 52%)
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cellular
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• a significant increase in apoptotic (TUNEL+) cells was observed in the cortex
• a significant increase in apoptotic (TUNEL+) cells was associated with the dilated tubules in the medulla
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• a significant increase in proliferative (Ki67+) cells was noted in the cortex, probably associated with proximal tubular structures, but not in the medulla
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growth/size/body
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• adult renal medullary collecting ducts exhibited cyst formation
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