Analysis Tools
mortality/aging
|
• 80% of mice fail to survive to adulthood
|
renal/urinary system
|
• by 5 months, kidneys exhibit characteristics of nephronophthisis with abnormal tubular basement membrane including thickening and disintegration with tubular collapse, interstitial cell infiltration and fibrosis, and multiple microcysts and tubular dilation unlike in wild-type mice
• the cortico-medullary region is most affected
• however, kidney ciliogenesis is normal
|
kidney cyst
(
J:154321
)
|
• by 5 months, mice exhibit microcysts unlike wild-type mice
• in mice older than 1 year of age, the average cyst area as a ration of total kidney area is increased compared to in younger mice
|
small kidney
(
J:154321
)
|
• by 5 months
|
|
• by 5 months
|
|
• by 5 months
|
|
• mice subjected to renal ischemia and reperfusion or cisplatin treatment exhibit defective recovery with increased cysts compared with similarly treated wild-type mice
• however, susceptibility to renal ischemia/reperfusion injury is normal
|
|
• by 5 months
|
isosthenuria
(
J:154321
)
|
• at 21 months, mice exhibit defective urine-concentrating ability compared to in wild-type mice
|
homeostasis/metabolism
|
• moderately at 21 months
|
|
• at 21 months
|
|
• mice subjected to renal ischemia and reperfusion or cisplatin treatment exhibit defective recovery compared with similarly treated wild-type mice
• however, susceptibility to renal ischemia/reperfusion- or cisplatin-induced injury is normal
|
immune system
|
• by 5 months
|
growth/size/body
kidney cyst
(
J:154321
)
|
• by 5 months, mice exhibit microcysts unlike wild-type mice
• in mice older than 1 year of age, the average cyst area as a ration of total kidney area is increased compared to in younger mice
|
cellular
|
• in kidneys at 5 months
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| nephronophthisis | DOID:12712 |
OMIM:PS256100 |
J:154321 | |
