mortality/aging
• about 80% die before weaning; however the lifespan of survivors is similar to wild-type
• injection of saline from P1 to 2 weeks of age rescues lethality in about 50% of mice
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renal/urinary system
• the wall of afferent arterioles is thickened and contains more cells
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• the wall of interlobular arteries is thickened and contains more cells
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• daily urinary excretion of aldosterone is less than 10% of wild-type mice
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• following 12 hours of water deprivation mice fail to concentrate their urine
• treatment with 1 ng/g desmopressin fails to increase urine osmolality unlike in wild-type mice
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• perivascular infiltration of mononuclear cells
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• focal
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• severely affected kidneys may display papillary atrophy
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• severely affected kidneys display atrophic or hypoplastic papilla
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• variable severity hydronephrosis develops after birth
• severely affected kidneys have a dilated renal pelvis
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small kidney
(
J:110082
)
• severely affected kidneys have a thinned medulla
|
• surface of severely affected kidneys appears granular
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• excrete about 5 times more urine daily; however daily urinary sodium, potassium, chloride and creatinine excretion are similar to wild-type
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cardiovascular system
• the wall of afferent arterioles is thickened and contains more cells
|
• the wall of interlobular arteries is thickened and contains more cells
|
• at 4 months of age blood pressure is about 30 mmHg lower than wild-type mice
• treatment with Angiotensin II restores blood pressure to normal
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homeostasis/metabolism
• at 4 months of age
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• at 4 months of age
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• angiotensin II is undetectable in plasma
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• renin and angiotensins I and II are undetectable in plasma
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dehydration
(
J:110082
)
• during 12 hours of water deprivation mice lose over 20% of their body weight
|
• metabolic acidosis at 4 months of age
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• daily urinary excretion of aldosterone is less than 10% of wild-type mice
|
• following 12 hours of water deprivation mice fail to concentrate their urine
• treatment with 1 ng/g desmopressin fails to increase urine osmolality unlike in wild-type mice
|
growth/size/body
• survivors weigh about 20% less than littermate controls
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reproductive system
N |
• survivors display normal fertility
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immune system
• perivascular infiltration of mononuclear cells
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