renal/urinary system
• mesangial cells show increased nuclear size and a prominent nucleolus
|
• mesangial hypercellularity
|
• microcysts form in the tubules along with protein casts
• cysts are lined by hypertrophic tubular epithelial cells
|
• proteinuria is detectable in some mice
|
• inflammatory infiltrates surround damaged blood vessels in the kidney
|
• juxtaglomerular hyperplasia is evident in diseased kidneys
|
• increased mesangial matrix leading to obliteration of the glomerular capillary bed
|
• glomerulosclerosis occurs in about half of females and a quarter of males by 15 months of age
• glomerulosclerosis incidence and severity increases with age
• the disease progresses from a focal and segmental sclerosis to a more diffuse and global pattern
• glomerular tuft collapse and hypertensive nephropathy also occur in the diseased kidney
|
renal cast
(
J:135449
)
• protein casts
|
homeostasis/metabolism
• proteinuria is detectable in some mice
|
immune system
• inflammatory infiltrates surround damaged blood vessels in the kidney
|
growth/size/body
• microcysts form in the tubules along with protein casts
• cysts are lined by hypertrophic tubular epithelial cells
|
cellular
• mesangial cells show increased nuclear size and a prominent nucleolus
|
• mesangial hypercellularity
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Denys-Drash syndrome | DOID:3764 |
OMIM:194080 |
J:135449 |