Analysis Tools
mortality/aging
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• only 29% of expected mice survive birth
• however, none of the mice with respiratory failure had enlarged or cystic kidneys and breeding subsequent generations from surviving homozygotes selectively eliminated prenatal and neonatal lethality
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• a subset of mice born succumbing to respiratory failure within the first day after birth
• however, none of the mice with respiratory failure had enlarged or cystic kidneys and breeding subsequent generations from surviving homozygotes selectively eliminated prenatal and neonatal lethality
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renal/urinary system
kidney cyst
(
J:125113
)
|
• mice develop varying degrees of renal cystic disease that worsens with age
• 13.7% of mice younger than 3 months of age display cysts with more mice exhibiting cysts as they age
• the number of mice with severe kidney cysts increases from 10% at a young age to 55% in older mice
• cysts radiate from the papilla to the cortex
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• kidney volume is increased due to the presence of cysts
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• mice exhibit dilated kidney tubules even when no cysts are present
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liver/biliary system
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• ductal plate malfomations develop with biliary duct proliferation, biliary cysts and mild to severe periportal fibrosis
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biliary cyst
(
J:125113
)
|
• all mice develop biliary cysts with some mice developing choledochal cysts and ascending cholangitis
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liver cyst
(
J:125113
)
|
• some mice develop ascending cholangitis
|
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• mice develop mild to severe periportal fibrosis
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respiratory system
|
• a subset of mice born succumbing to respiratory failure within the first day after birth
• however, none of the mice with respiratory failure had enlarged or cystic kidneys and breeding subsequent generations from surviving homozygotes selectively eliminated prenatal and neonatal lethality
|
growth/size/body
biliary cyst
(
J:125113
)
|
• all mice develop biliary cysts with some mice developing choledochal cysts and ascending cholangitis
|
|
• 33.3% of mice develop pancreatic cysts at older than 9 months of age
• severe pancreatic cysts are associated with choledochal cysts and massive dilation of the pancreatic duct (up to 6 cm)
• incidence of extra-renal phenotypes increases over time
|
kidney cyst
(
J:125113
)
|
• mice develop varying degrees of renal cystic disease that worsens with age
• 13.7% of mice younger than 3 months of age display cysts with more mice exhibiting cysts as they age
• the number of mice with severe kidney cysts increases from 10% at a young age to 55% in older mice
• cysts radiate from the papilla to the cortex
|
liver cyst
(
J:125113
)
|
• growth retardation occurs in some mice with mild extra-renal disease but proceeds it
|
|
• kidney volume is increased due to the presence of cysts
|
immune system
|
• some mice develop ascending cholangitis
|
endocrine/exocrine glands
|
• ductal plate malfomations develop with biliary duct proliferation, biliary cysts and mild to severe periportal fibrosis
|
biliary cyst
(
J:125113
)
|
• all mice develop biliary cysts with some mice developing choledochal cysts and ascending cholangitis
|
|
• 33.3% of mice develop pancreatic cysts at older than 9 months of age
• severe pancreatic cysts are associated with choledochal cysts and massive dilation of the pancreatic duct (up to 6 cm)
• incidence of extra-renal phenotypes increases over time
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal recessive polycystic kidney disease | DOID:0110861 | J:125113 | ||
