mortality/aging
• mice start dying from 26 weeks of age and 72.2% die by 30 weeks of age
|
growth/size/body
• body weight increases normally until 14 weeks of age but does not increase as much as in wild-type mice after 16 weeks of age
|
renal/urinary system
albuminuria
(
J:283613
)
• urinary albumin levels tend to be increased at 6 weeks of age and increase with age, remaining high after 22 weeks of age
• urinary albumin corrected by creatinine is increased with aging
|
• 22-week old mice show thickened Bowmans capsule
|
• 6-week old mice show occasional foot process effacement
|
• 6-week old mice show focal irregularity of glomerular basement membrane
|
• 22-week old mice show thickening with matrix lamination in glomerular basement membrane
|
• few glomeruli exhibit collapsed glomerular tuft accompanied by parietal cell hyperplasia mimicking crescent formation in 6-week old mice
• tubulointerstitial changes are limited to the peri-glomerular area of involved glomeruli
• marked glomerular changes are seen at 22 weeks, including glomerular tuft collapse with thickened Bowmans capsule
|
• 22-week old mice exhibit increased mesangial matrices
|
• tubulointerstitial changes are associated with accumulated glomerulosclerosis
|
• 6-week old mice exhibit tubulointerstitial fibrosis
|
homeostasis/metabolism
• increases with aging
|
• increases with aging
|
albuminuria
(
J:283613
)
• urinary albumin levels tend to be increased at 6 weeks of age and increase with age, remaining high after 22 weeks of age
• urinary albumin corrected by creatinine is increased with aging
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
X-linked Alport syndrome | DOID:0110034 |
OMIM:301050 |
J:283613 |