Analysis Tools
renal/urinary system
kidney cyst
(
J:140302
)
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• females begin to develop cystic dilation in the outer stripe of the outer medulla and inner cortex at around 9 months of age
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• in older female mice in dilated tubules the brush border is attenuated or in extreme cases lost
• in females at 9 months of age, surface blebs are seen on cilia and some cilia appear as branched clumps
• in female mice at 15 months of age cilia on the lining of cysts are usually stunted and highly branched
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• dilation is confined to the proximal tubules in females at 9 - 15 months of age
• dilation in females increases from 9 to 12 months of age
• Background Sensitivity: dilation occurs later on a mixed background compared to congenic mice on either BALB/cJ or C57BL/6J backgrounds
• dilation is not seen in males at up to 18 months of age
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liver/biliary system
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• primary cilia are shorter and often misshapen with multiple blebs in biliary trees affected by cystic changes
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• in early stages proliferation is seen in portal areas as primitive tortuous ducts and reactive stroma
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• seen in all mice by 1 month of age
• worsens with age
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liver cyst
(
J:140302
)
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• develop fibrocystic liver disease
• by 12 - 15 months of age, most of the liver is replaced with cysts
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• increase in the liver to body weight ratio by 9 months of age
• livers are often massively enlarged occupying much of the abdominal cavity
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• develop fibrocystic liver disease
• by 6 - 9 months of age, portal fibrosis is evident
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digestive/alimentary system
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• duct walls are thickened and lined with hyperplastic columnar epithelium
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• ducts are dilated
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endocrine/exocrine glands
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• primary cilia are shorter and often misshapen with multiple blebs in biliary trees affected by cystic changes
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• duct walls are thickened and lined with hyperplastic columnar epithelium
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• ducts are dilated
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• in early stages proliferation is seen in portal areas as primitive tortuous ducts and reactive stroma
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• seen in all mice by 1 month of age
• worsens with age
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• in some mice the pancreas is enlarged and markedly cystic
• dilated cysts are lined with hyperplastic columnar epithelium surrounded by fibrotic stroma
• microscopic cysts were seen in 44 of 92 mice
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cellular
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• primary cilia are shorter and often misshapen with multiple blebs in biliary trees affected by cystic changes
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growth/size/body
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• in some mice the pancreas is enlarged and markedly cystic
• dilated cysts are lined with hyperplastic columnar epithelium surrounded by fibrotic stroma
• microscopic cysts were seen in 44 of 92 mice
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kidney cyst
(
J:140302
)
|
|
• females begin to develop cystic dilation in the outer stripe of the outer medulla and inner cortex at around 9 months of age
|
liver cyst
(
J:140302
)
|
• develop fibrocystic liver disease
• by 12 - 15 months of age, most of the liver is replaced with cysts
|
|
• increase in the liver to body weight ratio by 9 months of age
• livers are often massively enlarged occupying much of the abdominal cavity
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal recessive polycystic kidney disease | DOID:0110861 |
OMIM:263200 |
J:140302 | |
