renal/urinary system
• kidney cells exhibit endoplasmic reticulum hyperplasia, protein inclusions, and cell necrosis unlike in Tg(Umod)416Lura mice
|
• tubular cysts in the cortico-medullary area after 24 weeks
|
• at 12 weeks, mice exhibit sparse tubular atrophy unlike Tg(Umod)416Lura mice
• from 24 weeks, mice exhibit progressive tubulo-interstitial damage with increasing fibrosis, tubular cysts in the cortico-medullary area, and damaged tubules with cells containing vesicular structures and dense material compared with Tg(Umod)416Lura mice
• from 24 weeks, mice exhibit tracts of increased thickness or multilayered tubular basement membrane or gaps of the tubular basement membrane unlike in Tg(Umod)416Lura mice
|
• at 12 weeks, mice exhibit sparse tubular atrophy unlike Tg(Umod)416Lura mice
|
• mild to moderate at 12 weeks of age
|
• kidney tubular cells exhibit cell necrosis unlike in Tg(Umod)416Lura mice
|
renal cast
(
J:161532
)
• at 12 weeks, some dilated tubules are filled with proteins casts
|
• mice exhibit increased kidney cell proliferation unlike Tg(Umod)416Lura mice
|
• mice exhibit progressive interstitial inflammation with increased cell proliferation unlike Tg(Umod)416Lura mice
• mice exhibit increased numbers of interstitial macrophage between 12 and 24 weeks, particularly in the renal medulla, unlike Tg(Umod)416Lura mice
|
isosthenuria
(
J:161532
)
• mice fail to concentrate urine when subjected to water deprivation unlike Tg(Umod)416Lura mice
|
• mild
|
homeostasis/metabolism
behavior/neurological
polydipsia
(
J:161532
)
immune system
• mice exhibit progressive interstitial inflammation with increased cell proliferation unlike Tg(Umod)416Lura mice
• mice exhibit increased numbers of interstitial macrophage between 12 and 24 weeks, particularly in the renal medulla, unlike Tg(Umod)416Lura mice
|
cellular
• mice exhibit increased kidney cell proliferation unlike Tg(Umod)416Lura mice
|
growth/size/body
• tubular cysts in the cortico-medullary area after 24 weeks
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
familial juvenile hyperuricemic nephropathy | DOID:0060062 |
OMIM:162000 OMIM:613092 OMIM:614227 |
J:161532 | |
kidney disease | DOID:557 | J:161532 |