Phenotypes for Wt1 MGI:3803666 MGI Mouse

renal/urinary system

• mesangial cells show increased nuclear size and a prominent nucleolus

increased mesangial cell number ( J:135449 )

• mesangial hypercellularity

kidney cortex cyst ( J:135449 )

• microcysts form in the tubules along with protein casts

• cysts are lined by hypertrophic tubular epithelial cells

increased urine protein level ( J:135449 )

• proteinuria is first detected at 54 days of age and occurs in all mice by 18 months of age

• proteinuria occurs after the onset of glomerulosclerosis

glomerulonephritis ( J:135449 )

• inflammatory infiltrates surround damaged blood vessels in the kidney

juxtaglomerular cell hyperplasia ( J:135449 )

• juxtaglomerular hyperplasia is evident in diseased kidneys

podocyte hypertrophy ( J:135449 )

expanded mesangial matrix ( J:135449 )

• increased mesangial matrix leading to obliteration of the glomerular capillary bed

glomerulosclerosis ( J:135449 )

• glomerulosclerosis occurs in about 20-25% of mice by 6 months of age

• onset of glomerulosclerosis occurs earlier than heterozygote mice on a mixed or outbred background

• 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 disease kidney

renal cast ( J:135449 )

• protein casts

homeostasis/metabolism