mortality/aging
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• median survival time is 65 weeks compared to 94.1 weeks for wild-type
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renal/urinary system
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• in cystic renal epithelia cells, apoptotic indices are gradually increased from ~13.55% at 5-8 months of age to ~21.02% at 9-12-months of age, unlike in wild-type cells (~2.86%)
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• at 5-8 months of age, ~2.86% nuclei of cystic renal epithelial cells are PCNA-positive relative to ~0.85% in wild-type cells; like in hepatic cysts, the % of PCNA-positive nuclei does not change significantly (~2.55%) with age
|
|
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• all have bilateral renal cysts
(J:47035)
• exhibit renal cyst formation by 6-10 weeks of age that progresses to cystic replacement of normal renal parenchyma later in life
(J:59314)
• numerous macroscopically visible cysts are scattered on the kidney surface at an early disease stage
(J:158354)
• total renal cystic volume decreases from ~ 40.67% of total kidney parenchyma at 5-8 months to ~31.04% at 9-12 months of age
(J:158354)
• primary cilia in cystic renal epithelial cells remain normal despite a tendency to be shorter
(J:158354)
• no significant fibrosis is noted around renal cysts in either age group
(J:158354)
• mice show a similar level of polycystic kidney disease as single Pkd1tm1.1Pcha homozygotes
(J:220561)
|
|
|
• mutant kidneys are enlarged with numerous macroscopically visible cysts
|
|
|
• at 5-8 months of age, mutant kidney weights represent ~2% of total body weight
(J:158354)
• by 9-12 months of age, mutant kidney weights account for ~2.6% of total body weight
(J:158354)
• kidney weight to body weight ratio is increased to the same extent as in single Pkd1tm1.1Pcha homozygotes
(J:220561)
|
liver/biliary system
|
|
• at 5-8 months of age, apoptotic indices in cystic cholangiocytes are increased to ~3.41% relative to ~0.56% in wild-type cholangiocytes, but do not change significantly with age (~3.86% at 9-12 months)
|
|
|
• cystic cholangiocytes lining intrahepatic bile ducts are enlarged with a thickened basement membrane and a significantly larger width (i.e. distance between two cell junctions) relative to wild-type cholangiocytes
• most cystic cholangiocytes are squamous in appearance
|
|
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• cystic cholangiocyte cilia are ~2-fold shorter than wild-type
• ~25% of primary cilia in cystic cholangiocytes appear malformed (e.g. bulbs at the ciliary tip)
|
|
|
• 3 of 6 have bile duct proliferation
(J:47035)
• at 5-8 months of age, ~18.77% nuclei of cystic cholangiocytes are PCNA-positive relative to ~1.12% in wild-type cholangiocytes; however, the % of PCNA-positive nuclei does not change significantly with age (~20.26% at 9-12 months)
(J:158354)
|
|
|
• all have 1-10 liver cysts
(J:47035)
• at 9-12 months of age, multiple fluid-filled cysts of different size and shapes are noted in the liver parenchyma
(J:158354)
• hepatic cysts posses microvilli and have well-developed cellular junctions
(J:158354)
• epithelial cells lining liver cysts stain positively for CK-19, suggesting that hepatic cysts arise from cholangiocytes
(J:158354)
• most hepatic cysts are lined by a single layer of cholangiocytes while ~10% of liver cysts are multilayered
(J:158354)
• total liver cystic volume increases from ~15.84% of total liver parenchyma at 5-8 months to ~23.26% at 9-12 months of age, as assessed by micro-CT scanning
(J:158354)
|
|
|
• mutant livers are significantly enlarged with multiple macroscopically visible cysts, often occupying most of the abdominal cavity
|
|
|
• at 5-8 months of age, mutant liver weights represent ~6% of total body weight relative to ~4.7% in wild-type controls
• by 9-12 months of age, mutant liver weights account for ~8% of total body weight, with no significant differences between male and female mice
|
|
|
• at 5-8 months of age, mild fibrotic deposits are observed around liver cysts, occupying ~2.71% of total liver parenchyma relative to ~0.92% in wild-type controls
• no changes in hepatic fibrosis are found during disease progression
|
homeostasis/metabolism
endocrine/exocrine glands
|
|
• cystic cholangiocytes lining intrahepatic bile ducts are enlarged with a thickened basement membrane and a significantly larger width (i.e. distance between two cell junctions) relative to wild-type cholangiocytes
• most cystic cholangiocytes are squamous in appearance
|
|
|
• cystic cholangiocyte cilia are ~2-fold shorter than wild-type
• ~25% of primary cilia in cystic cholangiocytes appear malformed (e.g. bulbs at the ciliary tip)
|
|
|
• 3 of 6 have bile duct proliferation
(J:47035)
• at 5-8 months of age, ~18.77% nuclei of cystic cholangiocytes are PCNA-positive relative to ~1.12% in wild-type cholangiocytes; however, the % of PCNA-positive nuclei does not change significantly with age (~20.26% at 9-12 months)
(J:158354)
|
|
|
• 50% of mutants over 3 months of age have visible cysts
|
cellular
|
|
• cystic cholangiocyte cilia are ~2-fold shorter than wild-type
• ~25% of primary cilia in cystic cholangiocytes appear malformed (e.g. bulbs at the ciliary tip)
|
|
|
• in cystic renal epithelia cells, apoptotic indices are gradually increased from ~13.55% at 5-8 months of age to ~21.02% at 9-12-months of age, unlike in wild-type cells (~2.86%)
|
|
|
• at 5-8 months of age, apoptotic indices in cystic cholangiocytes are increased to ~3.41% relative to ~0.56% in wild-type cholangiocytes, but do not change significantly with age (~3.86% at 9-12 months)
|
|
|
• at 5-8 months of age, ~2.86% nuclei of cystic renal epithelial cells are PCNA-positive relative to ~0.85% in wild-type cells; like in hepatic cysts, the % of PCNA-positive nuclei does not change significantly (~2.55%) with age
|
growth/size/body
|
|
• 50% of mutants over 3 months of age have visible cysts
|
|
|
• all have bilateral renal cysts
(J:47035)
• exhibit renal cyst formation by 6-10 weeks of age that progresses to cystic replacement of normal renal parenchyma later in life
(J:59314)
• numerous macroscopically visible cysts are scattered on the kidney surface at an early disease stage
(J:158354)
• total renal cystic volume decreases from ~ 40.67% of total kidney parenchyma at 5-8 months to ~31.04% at 9-12 months of age
(J:158354)
• primary cilia in cystic renal epithelial cells remain normal despite a tendency to be shorter
(J:158354)
• no significant fibrosis is noted around renal cysts in either age group
(J:158354)
• mice show a similar level of polycystic kidney disease as single Pkd1tm1.1Pcha homozygotes
(J:220561)
|
|
|
• all have 1-10 liver cysts
(J:47035)
• at 9-12 months of age, multiple fluid-filled cysts of different size and shapes are noted in the liver parenchyma
(J:158354)
• hepatic cysts posses microvilli and have well-developed cellular junctions
(J:158354)
• epithelial cells lining liver cysts stain positively for CK-19, suggesting that hepatic cysts arise from cholangiocytes
(J:158354)
• most hepatic cysts are lined by a single layer of cholangiocytes while ~10% of liver cysts are multilayered
(J:158354)
• total liver cystic volume increases from ~15.84% of total liver parenchyma at 5-8 months to ~23.26% at 9-12 months of age, as assessed by micro-CT scanning
(J:158354)
|
|
|
• mutant kidneys are enlarged with numerous macroscopically visible cysts
|
|
|
• at 5-8 months of age, mutant kidney weights represent ~2% of total body weight
(J:158354)
• by 9-12 months of age, mutant kidney weights account for ~2.6% of total body weight
(J:158354)
• kidney weight to body weight ratio is increased to the same extent as in single Pkd1tm1.1Pcha homozygotes
(J:220561)
|
|
|
• mutant livers are significantly enlarged with multiple macroscopically visible cysts, often occupying most of the abdominal cavity
|
|
|
• at 5-8 months of age, mutant liver weights represent ~6% of total body weight relative to ~4.7% in wild-type controls
• by 9-12 months of age, mutant liver weights account for ~8% of total body weight, with no significant differences between male and female mice
|
Analysis Tools