Phenotypes Associated with This Genotype

Genotype
MGI:4413583

• Allelic Composition: Wt1^tm1.1Lahe/Wt1⁺
• Genetic Background: involves: 129S2/SvPas * C57BL/6 * FVB/NCrl * SJL

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:154995 )

• average lifespan is 4.6 months

renal/urinary system

increased urine protein level ( J:154995 )

• Background Sensitivity: at 2 to 8 months, 6 of 9 male mice from the first generation (N1) on a background containing FVB exhibit heavy proteinuria unlike mice backcrossed to a C57BL/6 background for 4 generations

• all mice from N2, N3, and N4 generations on the FVB containing background develop proteinuria by 2 weeks and 2 months unlike wild-type mice

abnormal glomerular capsule parietal layer morphology ( J:154995 )

• parietal cells stain for anti-IgG and anti-IgM antibodies unlike in wild-type mice

abnormal podocyte morphology ( J:154995 )

♂ • in male mice, GBM thickening is sometimes associated with podocyte hypertrophy and vacuolation unlike in wild-type mice

podocyte hypertrophy ( J:154995 )

♂ • in male mice, GBM thickening is sometimes associated with podocyte hypertrophy

increased renal glomerulus basement membrane thickness ( J:154995 )

• by the onset of proteinuria, male mice develop a GBM thickening that is sometimes associated with podocyte hypertrophy and vacuolation unlike in wild-type mice

abnormal renal glomerulus morphology ( J:154995 )

• glomerular lesions are frequently more severe at the periphery of the cortex with enlarged deeper glomeruli that are less affected

abnormal mesangial cell morphology ( J:154995 )

• mesangial cells stain for anti-IgG and anti-IgM antibodies unlike in wild-type mice

expanded mesangial matrix ( J:154995 )

• mice develop mesangial slerosis unlike in wild-type mice

mesangiolysis ( J:154995 )

• mice develop mesangiolysis unlike in wild-type mice

glomerulosclerosis ( J:154995 )

• all mice develop glomerulosclerosis with tubular damages with female mice developing renal pathologies later than male mice

homeostasis/metabolism

increased urine protein level ( J:154995 )

• Background Sensitivity: at 2 to 8 months, 6 of 9 male mice from the first generation (N1) on a background containing FVB exhibit heavy proteinuria unlike mice backcrossed to a C57BL/6 background for 4 generations

• all mice from N2, N3, and N4 generations on the FVB containing background develop proteinuria by 2 weeks and 2 months unlike wild-type mice

cellular

abnormal mesangial cell morphology ( J:154995 )

• mesangial cells stain for anti-IgG and anti-IgM antibodies unlike in wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Denys-Drash syndrome		DOID:3764	OMIM:194080	J:154995