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Phenotypes Associated with This Genotype
Genotype
MGI:3803665
Allelic
Composition
Wt1tm1Mlh/Wt1+
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1tm1Mlh mutation (2 available); any Wt1 mutation (28 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• microcysts form in the tubules along with protein casts
• cysts are lined by hypertrophic tubular epithelial cells

renal/urinary system
• 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
• mesangial cells show increased nuclear size and a prominent nucleolus
• mesangial hypercellularity
• 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
• protein casts

homeostasis/metabolism
• proteinuria is detectable in some mice

immune system
• inflammatory infiltrates surround damaged blood vessels in the kidney

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Denys-Drash syndrome DOID:3764 OMIM:194080
J:135449


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
11/23/2021
MGI 6.17
The Jackson Laboratory