mortality/aging
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• all mice die by 9 months of age
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• survival rate of pups is 41.24%
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renal/urinary system
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• mice exhibit macroscopic cysts derived from the distal tubules and collecting ducts and thick ascending loop of Henle by 3 months of age
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• by 3 months of age, all mice display enlarged kidneys with macroscopic cysts in a pattern that is similar to late-stage kidney disease in Pkhd1tm1.1Ggg homozygotes
• kidney volume is enlarged by more than 50% of that observed in the oldest, severely affected Pkhd1tm1.1Ggg homozygotes
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• kidney collecting ducts are dilated in a large proportion of mice at birth
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liver/biliary system
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• hepatic abnormalities are more severe and develop at a younger age than in Pkhd1tm1.1Ggg homozygotes
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• liver cysts with fibrosis are often present on the first day after birth
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growth/size/body
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• pancreatic cysts are more severe and develop at a younger age than in Pkhd1tm1.1Ggg homozygotes
• pancreatic cysts with fibrosis are often present on the first day after birth
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• mice exhibit macroscopic cysts derived from the distal tubules and collecting ducts and thick ascending loop of Henle by 3 months of age
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• liver cysts with fibrosis are often present on the first day after birth
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• growth retardation is more severe and develops at a younger age than in Pkhd1tm1.1Ggg homozygotes
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• by 3 months of age, all mice display enlarged kidneys with macroscopic cysts in a pattern that is similar to late-stage kidney disease in Pkhd1tm1.1Ggg homozygotes
• kidney volume is enlarged by more than 50% of that observed in the oldest, severely affected Pkhd1tm1.1Ggg homozygotes
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endocrine/exocrine glands
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• pancreatic cysts are more severe and develop at a younger age than in Pkhd1tm1.1Ggg homozygotes
• pancreatic cysts with fibrosis are often present on the first day after birth
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