All Phenotypes Wt1<+> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Wt1⁺
wild type
MGI:2432392

Summary

18 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	\Wt1^tm1Mlh/\Wt1⁺	129P2/OlaHsd-Wt1^tm1Mlh	MGI:3803666
ht2	\Wt1^tm2Hst/\Wt1⁺	chimera involves: 129P2/OlaHsd * C57BL/6JLac * CBA/CaLac	MGI:3611443
ht3	\Wt1^tm1Mlh/\Wt1⁺	involves: 129P2/OlaHsd * C57BL/6	MGI:3803665
ht4	\Wt1^tm1Mlh/\Wt1⁺	involves: 129P2/OlaHsd * C57BL/6 * MF1	MGI:3803667
ht5	\Wt1^tm1Nhsn/\Wt1⁺	involves: 129S2/SvPas * C57BL/6	MGI:3722876
ht6	\Wt1^tm1.1Lahe/\Wt1⁺	involves: 129S2/SvPas * C57BL/6 * FVB/NCrl * SJL	MGI:4413583
ht7	\Wt1^tm1.1Lahe/\Wt1⁺	involves: 129S2/SvPas * C57BL/6 * SJL	MGI:4413582
ht8	\Wt1^tm2.1Vih/\Wt1⁺	involves: 129S7/SvEvBrd * C57BL/6	MGI:3690049
ht9	\Wt1^tm1Vih/\Wt1⁺	involves: 129S7/SvEvBrd * C57BL/6	MGI:3512787
ht10	\Wt1^tm1Asc/\Wt1⁺	involves: 129S7/SvEvBrd * C57BL/6	MGI:3044970
ht11	\Wt1^tm1Vih/\Wt1⁺	involves: 129S7/SvEvBrd * C57BL/6 * MF1	MGI:3512883
cn12	\Drosha^tm1Litt/\Drosha^tm1Litt \Wt1^{tm2(cre/ERT2)Wtp}/\Wt1⁺	involves: 129P2/OlaHsd * 129S4/SvJae	MGI:6887989
cn13	\Wt1^{tm1(EGFP/cre)Wtp}/\Wt1⁺ \Zfpm2^tm1Sho/\Zfpm2^tm2Sho	involves: 129S1/Sv * 129S4/SvJae * 129S7/SvEvBrd	MGI:3851402
cn14	\Col1a1^{tm2(tetO-LIN28B)Gqda}/\Col1a1^{tm3(tetO-Mirlet7g/Mir21)Gqda} \Gt(ROSA)26Sor^{tm1(rtTA,EGFP)Nagy}/\Gt(ROSA)26Sor⁺ \Wt1^{tm2(cre/ERT2)Wtp}/\Wt1⁺	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * C57BL/6	MGI:5638880
cn15	\Col1a1^{tm2(tetO-LIN28B)Gqda}/\Col1a1⁺ \Gt(ROSA)26Sor^{tm1(rtTA,EGFP)Nagy}/\Gt(ROSA)26Sor⁺ \Wt1^{tm2(cre/ERT2)Wtp}/\Wt1⁺	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * C57BL/6	MGI:5638793
cn16	\Gt(ROSA)26Sor^tm1Sho/\Gt(ROSA)26Sor⁺ \Wt1^{tm1(EGFP/cre)Wtp}/\Wt1⁺ \Zfpm2^tm1Sho/\Zfpm2^tm1Sho	involves: 129S1/Sv * 129S4/SvJaeSor	MGI:3851406
cn17	\Dis3l2^em2Jtm/\Dis3l2^em2Jtm \Wt1^{tm1(EGFP/cre)Wtp}/\Wt1⁺	involves: 129S4/SvJae * C57BL/6J	MGI:6286059
cx18	\Pax2^1Neu/\Pax2⁺ \Wt1^tm1Jae/\Wt1⁺	involves: 102 * 129S4/SvJae * C57BL/6	MGI:3841045

Allelic Composition	\Wt1^tm1Mlh/\Wt1⁺
Genetic Background	129P2/OlaHsd-Wt1^tm1Mlh

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1Mlh mutation (2 available); any Wt1 mutation (34 available)

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

renal/urinary system

abnormal mesangial cell morphology ( J:135449 )

• 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

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

immune system

glomerulonephritis ( J:135449 )

• inflammatory infiltrates surround damaged blood vessels in the kidney

growth/size/body

kidney cortex cyst ( J:135449 )

• microcysts form in the tubules along with protein casts

• cysts are lined by hypertrophic tubular epithelial cells

cellular

abnormal mesangial cell morphology ( J:135449 )

• mesangial cells show increased nuclear size and a prominent nucleolus

increased mesangial cell number ( J:135449 )

• mesangial hypercellularity

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

Allelic Composition	\Wt1^tm2Hst/\Wt1⁺
Genetic Background	chimera involves: 129P2/OlaHsd * C57BL/6JLac * CBA/CaLac

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm2Hst mutation (0 available); any Wt1 mutation (34 available)

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

reproductive system

azoospermia ( J:103489 )

• the XXY heterozygous male had aspermic epididymides

small testis ( J:53585 , J:103489 )

(J:53585)

(J:103489)

abnormal reproductive system physiology ( J:103489 )

• male sex reversal does not occur, however, only 2 of 23 XX to XY chimeras developed as phenotypic males, a significantly lower proportion than the 40% found in wild-type XX to XY chimeras, indicating that the sex ratio is distorted

male infertility ( J:53585 )

• a single heterozygous male, that is 41, XXY, was obtained from a female chimera and is sterile

renal/urinary system

abnormal kidney vasculature morphology ( J:53585 )

• kidneys of the heterozygous male exhibit a secondary hypertensive nephropathy, indicating kidney disease

abnormal kidney interlobular artery morphology ( J:53585 )

• exhibit interlobular arteries with severe hypertensive damage, including fibrinoid necrosis, medial hypertrophy and hyperplasia, and loss of the arterial lumen

abnormal glomerular capillary morphology ( J:53585 )

• obliteration of the glomerular capillary bed

abnormal mesangial cell morphology ( J:53585 )

• mesangial cells show an increase in nuclear size, a prominent nucleolus, and hypercellularity

increased mesangial cell number ( J:53585 )

• mesangial hypercellularity

kidney cyst ( J:53585 )

• kidneys of the heterozygous male exhibit tubular epithelial microcyst formation

decreased glomerular capsule space ( J:53585 )

• obliteration of the urinary filtration space

absent podocyte foot process ( J:53585 )

• podocytes show loss of foot processes

podocyte hypertrophy ( J:53585 )

• podocyte hypertrophy and hyperplasia

podocyte microvillus transformation ( J:53585 )

• podocytes show microvillus transformation of the apical surface

expanded mesangial matrix ( J:53585 )

• kidneys of the heterozygous male and of chimeras, although to a lesser extent than the heterozygote, exhibit a diffuse and global mesangial sclerosis, indicating kidney disease

glomerulosclerosis ( J:53585 )

• global sclerosis of the glomerular tuft and obliteration of the glomerular capillary bed and urinary filtration space by an increase in extracellular mesangial matrix

glomerular crescent ( J:53585 )

• glomerulus shows focal crescent formation

renal interstitial fibrosis ( J:53585 )

dilated renal tubule ( J:53585 )

kidney failure ( J:53585 )

• the male heterozygote exhibits end-stage renal failure at 8 months of age

endocrine/exocrine glands

small testis ( J:53585 , J:103489 )

(J:53585)

(J:103489)

cardiovascular system

abnormal kidney vasculature morphology ( J:53585 )

• kidneys of the heterozygous male exhibit a secondary hypertensive nephropathy, indicating kidney disease

abnormal kidney interlobular artery morphology ( J:53585 )

• exhibit interlobular arteries with severe hypertensive damage, including fibrinoid necrosis, medial hypertrophy and hyperplasia, and loss of the arterial lumen

abnormal glomerular capillary morphology ( J:53585 )

• obliteration of the glomerular capillary bed

cellular

azoospermia ( J:103489 )

• the XXY heterozygous male had aspermic epididymides

abnormal mesangial cell morphology ( J:53585 )

• mesangial cells show an increase in nuclear size, a prominent nucleolus, and hypercellularity

increased mesangial cell number ( J:53585 )

• mesangial hypercellularity

growth/size/body

kidney cyst ( J:53585 )

• kidneys of the heterozygous male exhibit tubular epithelial microcyst formation

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Denys-Drash syndrome		DOID:3764	OMIM:194080	J:53585 , J:103489

Allelic Composition	\Wt1^tm1Mlh/\Wt1⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1Mlh mutation (2 available); any Wt1 mutation (34 available)

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

renal/urinary system

abnormal mesangial cell morphology ( J:135449 )

• 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 detectable in some mice

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 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

increased urine protein level ( J:135449 )

• proteinuria is detectable in some mice

immune system

glomerulonephritis ( J:135449 )

• inflammatory infiltrates surround damaged blood vessels in the kidney

growth/size/body

kidney cortex cyst ( J:135449 )

• microcysts form in the tubules along with protein casts

• cysts are lined by hypertrophic tubular epithelial cells

cellular

abnormal mesangial cell morphology ( J:135449 )

• mesangial cells show increased nuclear size and a prominent nucleolus

increased mesangial cell number ( J:135449 )

• mesangial hypercellularity

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

Allelic Composition	\Wt1^tm1Mlh/\Wt1⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * MF1

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1Mlh mutation (2 available); any Wt1 mutation (34 available)

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

renal/urinary system

abnormal mesangial cell morphology ( J:135449 )

• 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 detectable in some mice

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 half of 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 disease kidney

renal cast ( J:135449 )

• protein casts

homeostasis/metabolism

increased urine protein level ( J:135449 )

• proteinuria is detectable in some mice

immune system

glomerulonephritis ( J:135449 )

• inflammatory infiltrates surround damaged blood vessels in the kidney

cellular

abnormal mesangial cell morphology ( J:135449 )

• mesangial cells show increased nuclear size and a prominent nucleolus

increased mesangial cell number ( J:135449 )

• mesangial hypercellularity

growth/size/body

kidney cortex cyst ( J:135449 )

• microcysts form in the tubules along with protein casts

• cysts are lined by hypertrophic tubular epithelial cells

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

Allelic Composition	\Wt1^tm1Nhsn/\Wt1⁺
Genetic Background	involves: 129S2/SvPas * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1Nhsn mutation (1 available); any Wt1 mutation (34 available)

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

normal phenotype

no abnormal phenotype detected ( J:123972 )

• heterozygous mice are indistinguishable with respect to hematopoietic cellularity and lineage composition from wild-type littermates

• loss of a single allele did not affect viability and expected Mendelian ratio of heterozygous mice are born by heterozygous crosses

• used for expression study only

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1.1Lahe mutation (0 available); any Wt1 mutation (34 available)

♀	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 )

• 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

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1.1Lahe mutation (0 available); any Wt1 mutation (34 available)

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

renal/urinary system

renal/urinary system phenotype ( J:154995 )

N	• mice exhibit normal kidney morphology and function unlike mice on a background containing FVB

Allelic Composition	\Wt1^tm2.1Vih/\Wt1⁺
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm2.1Vih mutation (0 available); any Wt1 mutation (34 available)

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

normal phenotype

no abnormal phenotype detected ( J:111787 )

• mice are phenotypically normal and viable

Allelic Composition	\Wt1^tm1Vih/\Wt1⁺
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1Vih mutation (0 available); any Wt1 mutation (34 available)

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

reproductive system

impaired granulosa cell differentiation ( J:204518 )

• at 3 weeks of age, 3beta-HSD protein is detected not only in the interstitium (as in control ovaries) but also in the granulosa cells of small follicles (unlike in control ovaries); also, mRNA expression of LHR, aromatase and FSHR is increased whereas mRNA expression of Amh is reduced, suggesting premature differentiation of granulosa cells

abnormal granulosa cell morphology ( J:204518 )

• at 3 weeks of age, many developing follicles show asymmetric granulosa cells or no granulosa cells with cuboidal morphology

impaired ovarian folliculogenesis ( J:204518 )

• at 1 week of age, the total number of developing follicles with multiple layers of granulosa cells is significantly lower than that in control ovaries (~44 versus ~205, respectively)

• at 3 weeks of age, the number of developing follicles is significantly reduced and many aberrant follicles with undifferentiated squamous granulosa cells are observed

• when follicles with 2-3 layers of granulosa cells are obtained from 2-week-old ovaries and cultured in vitro, only ~11% of follicles develop to the pre-ovulatory stage and most follicles are relatively small or exhibit detached oocytes after 11 days in culture, whereas ~80% of control follicles develop to the preovulatory stage with a notable antrum

• aberrant follicle development is likely due to premature differentiation and abnormal cell polarity establishment of granulosa cells

• however, granulosa cell proliferation is normal, as shown by Ki67 staining

abnormal primary ovarian follicle morphology ( J:204518 )

• at 1 week of age, many primary follicles with asymmetric and disorganized granulosa cells are observed

small ovary ( J:204518 )

• ovary size is dramatically reduced at 3 weeks of age

decreased ovulation rate ( J:204518 )

• ovulation rate of females is severely reduced with an average of only 2 oocytes obtained by natural ovulation versus 8 oocytes in control females

decreased superovulation rate ( J:204518 )

• after hormone stimulation, 3-week-old females release less than 5 oocytes versus ~64 oocytes released from control females

reduced female fertility ( J:204518 )

• only ~15% of plugged females become pregnant after mating with wild-type males versus 88.2% of control females

cellular

impaired granulosa cell differentiation ( J:204518 )

endocrine/exocrine glands

impaired granulosa cell differentiation ( J:204518 )

abnormal granulosa cell morphology ( J:204518 )

• at 3 weeks of age, many developing follicles show asymmetric granulosa cells or no granulosa cells with cuboidal morphology

impaired ovarian folliculogenesis ( J:204518 )

• at 1 week of age, the total number of developing follicles with multiple layers of granulosa cells is significantly lower than that in control ovaries (~44 versus ~205, respectively)

• at 3 weeks of age, the number of developing follicles is significantly reduced and many aberrant follicles with undifferentiated squamous granulosa cells are observed

• aberrant follicle development is likely due to premature differentiation and abnormal cell polarity establishment of granulosa cells

• however, granulosa cell proliferation is normal, as shown by Ki67 staining

abnormal primary ovarian follicle morphology ( J:204518 )

• at 1 week of age, many primary follicles with asymmetric and disorganized granulosa cells are observed

small ovary ( J:204518 )

• ovary size is dramatically reduced at 3 weeks of age

renal/urinary system

renal/urinary system phenotype ( J:94225 )

N	• no proteinuria or glomerulosclerosis detected unlike on a mixed 129S7/SvEvBrd, C57BL/6, and MF1 background

Allelic Composition	\Wt1^tm1Asc/\Wt1⁺
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1Asc mutation (0 available); any Wt1 mutation (34 available)

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

mortality/aging

neonatal lethality, complete penetrance ( J:71149 )

• death due to kidney defects within 24 hrs after birth

renal/urinary system

abnormal renal glomerulus morphology ( J:71149 )

• increase in stromal tissue and decrease in tubular epithelium

small kidney ( J:71149 )

• characterized by localized macroscopic hemorrhage

reproductive system

decreased germ cell number ( J:71149 )

• germ cells are fewer and abnormally organized

primary sex reversal ( J:71149 )

• gonads of XY mice are ovarian-like and cryptorchid

cellular

decreased germ cell number ( J:71149 )

• germ cells are fewer and abnormally organized

Allelic Composition	\Wt1^tm1Vih/\Wt1⁺
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * MF1

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^tm1Vih mutation (0 available); any Wt1 mutation (34 available)

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

mortality/aging

premature death ( J:94225 )

• several mice died at 4 to 5 months of age

homeostasis/metabolism

increased urine protein level ( J:94225 )

• 100% of males and 43% of females at 4 months of age and 71% of females at 10 to 12 months of age displayed proteinuria

renal/urinary system

increased urine protein level ( J:94225 )

• 100% of males and 43% of females at 4 months of age and 71% of females at 10 to 12 months of age displayed proteinuria

podocyte foot process effacement ( J:94225 )

• effacement of podocyte foot processes

glomerulosclerosis ( J:94225 )

• 33% of males and 0% of females by 2 months of age and 100% of males and 43% of females at 4 months of age displayed glomerulosclerosis

• medullary regions of the kidneys had dilated tubules and protein casts, a few glomeruli with segmental sclerosis, and predominately glomeruli with global mesangial sclerosis

• glomeruli displayed severe thickening of the glomerular basement membrane

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Denys-Drash syndrome		DOID:3764	OMIM:194080	J:94225

Allelic Composition	\Drosha^tm1Litt/\Drosha^tm1Litt \Wt1^{tm2(cre/ERT2)Wtp}/\Wt1⁺
Genetic Background	involves: 129P2/OlaHsd * 129S4/SvJae

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Drosha^tm1Litt mutation (3 available); any Drosha mutation (98 available)
Wt1^{tm2(cre/ERT2)Wtp} mutation (1 available); any Wt1 mutation (34 available)

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

renal/urinary system

renal/urinary system phenotype ( J:321428 )

N	• normal kidney size with fully developed glomeruli

increased urine protein level ( J:321428 )

• protein deposits in kidney tubules by age P28

homeostasis/metabolism

increased urine protein level ( J:321428 )

• protein deposits in kidney tubules by age P28

neoplasm

neoplasm ( J:321428 )

N	• no kidney tumor development in mice up to 6 months old

mortality/aging

mortality/aging ( J:321428 )

N	• viable; live to at least 6 months

Allelic Composition	\Wt1^{tm1(EGFP/cre)Wtp}/\Wt1⁺ \Zfpm2^tm1Sho/\Zfpm2^tm2Sho
Genetic Background	involves: 129S1/Sv * 129S4/SvJae * 129S7/SvEvBrd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^{tm1(EGFP/cre)Wtp} mutation (1 available); any Wt1 mutation (34 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (45 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (45 available)

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

mortality/aging

perinatal lethality, complete penetrance ( J:150452 )

• pups die in perinatal period

cardiovascular system

cardiovascular system phenotype ( J:150452 )

N	• coronary vascular development is not impaired

thin myocardium compact layer ( J:150452 )

• compact myocardium is thin

abnormal atrioventricular cushion morphology ( J:150452 )

• severe atrio-ventricular endocardial cushion defect is observed by E14.5

muscle

thin myocardium compact layer ( J:150452 )

• compact myocardium is thin

Allelic Composition	\Col1a1^{tm2(tetO-LIN28B)Gqda}/\Col1a1^{tm3(tetO-Mirlet7g/Mir21)Gqda} \Gt(ROSA)26Sor^{tm1(rtTA,EGFP)Nagy}/\Gt(ROSA)26Sor⁺ \Wt1^{tm2(cre/ERT2)Wtp}/\Wt1⁺
Genetic Background	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Col1a1^{tm2(tetO-LIN28B)Gqda} mutation (1 available); any Col1a1 mutation (166 available)
Col1a1^{tm3(tetO-Mirlet7g/Mir21)Gqda} mutation (1 available); any Col1a1 mutation (166 available)
Gt(ROSA)26Sor^{tm1(rtTA,EGFP)Nagy} mutation (5 available); any Gt(ROSA)26Sor mutation (1097 available)
Wt1^{tm2(cre/ERT2)Wtp} mutation (1 available); any Wt1 mutation (34 available)

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

renal/urinary system

small kidney ( J:211179 )

• small kidney in mice induced with doxycycline from E14.5 until the end of the experiment

• however, cap mesenchyme differentiates normally in doxycycline induced mutants

Allelic Composition	\Col1a1^{tm2(tetO-LIN28B)Gqda}/\Col1a1⁺ \Gt(ROSA)26Sor^{tm1(rtTA,EGFP)Nagy}/\Gt(ROSA)26Sor⁺ \Wt1^{tm2(cre/ERT2)Wtp}/\Wt1⁺
Genetic Background	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Col1a1^{tm2(tetO-LIN28B)Gqda} mutation (1 available); any Col1a1 mutation (166 available)
Gt(ROSA)26Sor^{tm1(rtTA,EGFP)Nagy} mutation (5 available); any Gt(ROSA)26Sor mutation (1097 available)
Wt1^{tm2(cre/ERT2)Wtp} mutation (1 available); any Wt1 mutation (34 available)

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

neoplasm

increased kidney tumor incidence ( J:211179 )

• all mice develop kidney tumors within the first 2 weeks of life following doxycycline (Dox) induction during embryonic development at E0, E14.5 or E18.5

• tumors resemble Wilms tumor

• mice treated with doxycycline at P10 do not develop tumors

renal/urinary system

kidney cyst ( J:211179 )

• mice treated with doxycycline at P10 develop cystic kidneys

increased kidney tumor incidence ( J:211179 )

• all mice develop kidney tumors within the first 2 weeks of life following doxycycline (Dox) induction during embryonic development at E0, E14.5 or E18.5

• tumors resemble Wilms tumor

• mice treated with doxycycline at P10 do not develop tumors

abnormal kidney mesenchyme morphology ( J:211179 )

• doxycycline induced mice exhibit persistent proliferation of cap mesenchyme cells in adults

• however, cap mesenchyme cells within tumors retain a differentiation capacity that recapitulates normal kidney development

delayed kidney development ( J:211179 )

• timing of kidney development is prolonged in doxycycline induced mice, with sustaining proliferation of the cap mesenchyme cells into adulthood

growth/size/body

kidney cyst ( J:211179 )

• mice treated with doxycycline at P10 develop cystic kidneys

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
nephroblastoma		DOID:2154	OMIM:194070	J:211179

Allelic Composition	\Gt(ROSA)26Sor^tm1Sho/\Gt(ROSA)26Sor⁺ \Wt1^{tm1(EGFP/cre)Wtp}/\Wt1⁺ \Zfpm2^tm1Sho/\Zfpm2^tm1Sho
Genetic Background	involves: 129S1/Sv * 129S4/SvJaeSor

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^tm1Sho mutation (4 available); any Gt(ROSA)26Sor mutation (1097 available)
Wt1^{tm1(EGFP/cre)Wtp} mutation (1 available); any Wt1 mutation (34 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (45 available)

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

cardiovascular system

cardiovascular system phenotype ( J:150452 )

N	• mice show normal numbers of epicardium-derived cells (EPDCs) and epicardial epithelial-mesenchymal transition (EMT) occurs normally

Allelic Composition	\Dis3l2^em2Jtm/\Dis3l2^em2Jtm \Wt1^{tm1(EGFP/cre)Wtp}/\Wt1⁺
Genetic Background	involves: 129S4/SvJae * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dis3l2^em2Jtm mutation (1 available); any Dis3l2 mutation (58 available)
Wt1^{tm1(EGFP/cre)Wtp} mutation (1 available); any Wt1 mutation (34 available)

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

renal/urinary system

renal/urinary system phenotype ( J:272435 )

N	• mice exhibit normal kidney histology without evidence of malignancy up to at least 6 months of age

Allelic Composition	\Pax2^1Neu/\Pax2⁺ \Wt1^tm1Jae/\Wt1⁺
Genetic Background	involves: 102 * 129S4/SvJae * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pax2^1Neu mutation (1 available); any Pax2 mutation (44 available)
Wt1^tm1Jae mutation (3 available); any Wt1 mutation (34 available)

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

mortality/aging

lethality throughout fetal growth and development, incomplete penetrance ( J:86661 )

• fewer than expected mice are observed at E16

postnatal lethality, incomplete penetrance ( J:86661 )

• 49% fewer than expected mice survive beyond weaning compared

renal/urinary system

increased kidney apoptosis ( J:86661 )

• at E16, whole kidney apoptosis is increased compared to in wild-type mice

abnormal kidney collecting duct morphology ( J:86661 )

• the volume of collecting ducts is reduced compared to in wild-type mice

decreased renal glomerulus number ( J:86661 )

• compared to in Pax2^1Neu heterozygotes and wild-type mice

abnormal kidney development ( J:86661 )

• at E16 and E19, mice exhibit abnormal kidney development that is more severe than in Pax2^1Neu heterozygotes

• however, kidney development at E13 is normal

kidney medulla hypoplasia ( J:86661 )

abnormal kidney calyx morphology ( J:86661 )

• renal calyces are reduced in size and number compared to in wild-type mice

small kidney ( J:86661 )

• the left kidney is 48% smaller than normal while the right kidney is 58% smaller than normal

decreased nephron number ( J:86661 )

• kidneys contain fewer than normal nephrons with fewer mesenchymal condensates, comma-shaped bodies, and S-shaped bodies and looser mesenchyme adjacent to ureteric bud structures compared to in wild-type kidneys

absent kidney ( J:86661 )

• in 20% of mice

cellular

increased kidney apoptosis ( J:86661 )

• at E16, whole kidney apoptosis is increased compared to in wild-type mice

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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