cardiovascular system
• blood pressure is significantly reduced in homozygous females but not in males
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homeostasis/metabolism
• homozygous males have significantly elevated BUN however in homozygous females BUN is slightly but not significantly elevated
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• potassium levels are increased in both homozygous males and females
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• kidney renin concentrations are reduced to 10-20% of wild-type and plasma renin concentrations reduced to 1% and 30% of wild-type in homozygous females and males, respectively
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renal/urinary system
• undifferentiated cells are seen adjacent to the tubule in the outer cortex
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• only rare, small and thin, renin positive cells are seen in homozygotes and the number of renin positive cells does not increase after treatment with the ACE inhibitor captopril unlike in wild-type and heterozygous mice
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• about 25% of glomeruli show damage with 8.3% enlarged and hypercellular and 16% shrunken
• alpha-SMA positive cells are seen within the glomeruli similar to immature kidneys, however the thickness of the afferent arterioles and interlobular arteries is similar to wild-type
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• increased collagen deposition is seen in the glomeruli
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• 8.3% of glomeruli are enlarged and hypercellular
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• increased collagen deposition is seen in some interstitial areas
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• in severely affected kidneys the border between the inner and outer medullary bands is absent
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small kidney
(
J:95786
)
• kidneys are smaller than wild-type
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• kidneys weigh significantly less compared to wild-type
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• in severe cases kidneys appear shrunken
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• 16% of glomeruli appear shrunken; mainly located in areas containing abnormal tubules
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• in severely affected kidneys the cortex is thinner than normal
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• tubular atrophy is seen in areas of the cortex
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• reduced brush borders are seen on some proximal tubules
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• tubular dilation is seen in areas of the cortex
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• in severe cases kidneys appear shrunken without the normal shiny appearance and with a granular aspect and white fat patches
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