liver/biliary system
liver cyst
(
J:122291
)
• at 4 and 5 months, 50% of mice have numerous cysts
• cysts are susceptible to rupture and exude a clear and acellular fluid with occasional brown and rarely green tinge
|
• at 3 to 5 months, liver weight is increased even in cyst-free livers
|
• mice have an increased number of cells within the liver due to biliary epithelial cell hyperplasia, accompanied by oval cell proliferation, and small basophilic mononuclear cells
|
• biliary tract hyperplasia
|
• the liver portal triad shows increased fibrosis that diminishes the sinusoidal area
|
• liver proteoglycans are reduced
• however, liver development and function are normal
|
renal/urinary system
kidney cyst
(
J:122291
)
• basement membrane cysts in renal tubules are similar to those found in Cys1
|
• at 3, 10 months, kidney weight is increased
|
• at 6 to 7 months in 6 of 8 mice and 9 to 10 months in 5 of 7 mice
|
• at 9 to 10 months, males have dilated renal tubules
|
homeostasis/metabolism
• blood urea nitrogen is increased 21% in younger mice and 36% in older mice
|
growth/size/body
kidney cyst
(
J:122291
)
• basement membrane cysts in renal tubules are similar to those found in Cys1
|
liver cyst
(
J:122291
)
• at 4 and 5 months, 50% of mice have numerous cysts
• cysts are susceptible to rupture and exude a clear and acellular fluid with occasional brown and rarely green tinge
|
• at 3, 10 months, kidney weight is increased
|
• at 3 to 5 months, liver weight is increased even in cyst-free livers
|
• mice have an increased number of cells within the liver due to biliary epithelial cell hyperplasia, accompanied by oval cell proliferation, and small basophilic mononuclear cells
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
polycystic kidney disease | DOID:0080322 |
OMIM:PS173900 |
J:122291 |