129P2/OlaHsd-Wt1^tm1Mlh/H

Status	Available to order
EMMA ID	EM:04835
International strain name	129P2/OlaHsd-Wt1^tm1Mlh/H
Alternative name	Wt1(tmT396)
Strain type	Targeted Mutant Strains : Knock-in
Allele/Transgene symbol	Wt1^tm1Mlh
Gene/Transgene symbol	Wt1

Information from provider

Provider	Colin Miles
Provider affiliation	Institute of Human Genetics, Newcastle University
Genetic information	Targeted mutation truncating Wilms' tumour gene (Wt1) within exon 9. Similar to mutations associated with Denys-Drash syndrome.
Phenotypic information	Heterozygous mutant mice develop glomerulosclerosis (typical of Denys-Drash syndrome) with age.
Breeding history	Pure 129/Ola background strain produced from directly backcrossing chimaeric males with 129/Ola females. Heterozygous females are not fertile, so line is maintained through heterozygous males.
References	Effects on kidney disease, fertility and development in mice inheriting a protein-truncating Denys-Drash syndrome allele (Wt1tmT396).;Patek Charles E, Brownstein David G, Fleming Stewart, Wroe Caroline, Rose Lorraine, Webb Anna, Berry Rachel L, Devenney Paul S, Walker Marion, Maddocks Oliver D K, Lawrence Nicola J, Harrison David J, Wood Katrina M, Miles Colin G, Hooper Martin L, ;2008;Transgenic research;17;459-75; 18040647
Homozygous fertile	no
Homozygous viable	no
Homozygous matings required	no
Immunocompromised	not known

Information from EMMA

Archiving centre	Mary Lyon Centre at MRC Harwell, Oxford, United Kingdom
Animals used for archiving	heterozygous 129P2/OlaHsd
Breeding at archiving centre	Males were archived upon arrival. No breeding was performed at the archiving centre.

Disease and phenotype information

MGI allele-associated human disease models

Denys-Drash syndrome / DOID:3764

Orphanet associated rare diseases, based on orthologous gene matching

Nephroblastoma / Orphanet_654
Denys-Drash syndrome / Orphanet_220
Meacham syndrome / Orphanet_3097
Frasier syndrome / Orphanet_347
Genetic steroid-resistant nephrotic syndrome / Orphanet_656

MGI phenotypes (allele matching)

kidney cortex cysts / MGI
glomerulonephritis / MGI
increased urine protein level / MGI
glomerulosclerosis / MGI
abnormal mesangial cell morphology / MGI
juxtaglomerular cell hyperplasia / MGI
expanded mesangial matrix / MGI
renal cast / MGI
mesangial cell hyperplasia / MGI
podocyte hypertrophy / MGI
abnormal heart development / MGI
abnormal heart shape / MGI
abnormal kidney development / MGI
abnormal liver development / MGI
liver hypoplasia / MGI
decreased embryo size / MGI
absent ovary / MGI
hemopericardium / MGI
agonadal / MGI
absent testes / MGI
myocardium hypoplasia / MGI
lethality throughout fetal growth and development, complete penetrance / MGI
embryonic lethality during organogenesis, incomplete penetrance / MGI
absent metanephros / MGI
abnormal diaphragm development / MGI
abnormal heart apex morphology / MGI
abnormal adrenal gland development / MGI

MGI phenotypes (gene matching)

abnormal heart development / MGI
abnormal heart shape / MGI
kidney cortex cysts / MGI
abnormal kidney development / MGI
abnormal kidney blood vessel morphology / MGI
abnormal liver development / MGI
liver hypoplasia / MGI
absent spleen / MGI
small testis / MGI
abnormal lung development / MGI
pulmonary hypoplasia / MGI
decreased embryo size / MGI
edema / MGI
abnormal reproductive system physiology / MGI
male infertility / MGI
prenatal lethality / MGI
premature death / MGI
abnormal embryonic tissue morphology / MGI
no abnormal phenotype detected / MGI
small heart / MGI
decreased germ cell number / MGI
abnormal secondary sex determination / MGI
streak gonad / MGI
dilated renal tubules / MGI
glomerulonephritis / MGI
increased urine protein level / MGI
small kidney / MGI
primary sex reversal / MGI
renal interstitial fibrosis / MGI
absent ovary / MGI
kidney failure / MGI
kidney cysts / MGI
diaphragmatic hernia / MGI
azoospermia / MGI
hemopericardium / MGI
glomerulosclerosis / MGI
absent adrenal gland / MGI
abnormal renal glomerulus morphology / MGI
abnormal podocyte morphology / MGI
abnormal mesangial cell morphology / MGI
renal/urinary system phenotype / MGI
cardiovascular system phenotype / MGI
agonadal / MGI
abnormal mesenchyme morphology / MGI
absent testes / MGI
absent podocyte foot process / MGI
podocyte foot process effacement / MGI
increased splenocyte apoptosis / MGI
abnormal spleen development / MGI
myocardium hypoplasia / MGI
primary atelectasis / MGI
neonatal lethality, complete penetrance / MGI
perinatal lethality, complete penetrance / MGI
embryonic lethality, complete penetrance / MGI
lethality throughout fetal growth and development, complete penetrance / MGI
prenatal lethality, incomplete penetrance / MGI
embryonic lethality during organogenesis, incomplete penetrance / MGI
juxtaglomerular cell hyperplasia / MGI
abnormal kidney interlobular artery morphology / MGI
abnormal glomerular capillary morphology / MGI
expanded mesangial matrix / MGI
absent metanephros / MGI
increased metanephric mesenchyme apoptosis / MGI
renal cast / MGI
increased renal glomerulus basement membrane thickness / MGI
abnormal gonadal ridge morphology / MGI
mesangial cell hyperplasia / MGI
mesangiolysis / MGI
abnormal glomerular capsule parietal layer morphology / MGI
decreased glomerular capsule space / MGI
glomerular crescent / MGI
podocyte microvillus transformation / MGI
podocyte hypertrophy / MGI
abnormal diaphragm development / MGI
abnormal pleuropericardial membrane morphology / MGI
abnormal heart apex morphology / MGI
abnormal adrenal gland development / MGI

Literature references

Effects on kidney disease, fertility and development in mice inheriting a protein-truncating Denys-Drash syndrome allele (Wt1tmT396).;Patek Charles E, Brownstein David G, Fleming Stewart, Wroe Caroline, Rose Lorraine, Webb Anna, Berry Rachel L, Devenney Paul S, Walker Marion, Maddocks Oliver D K, Lawrence Nicola J, Harrison David J, Wood Katrina M, Miles Colin G, Hooper Martin L, ;2008;Transgenic research;17;459-75; 18040647

Information on how we integrate external resources can be found here

Order

Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740^*
Rederivation of mice from frozen stock, delivery time available upon request . €3880^*
Tissue - Types of tissue, service fee and delivery time available upon request

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

^* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

More details on pricing and delivery times

Practical information

Example health report
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Material Transfer Agreement (MTA)
MTA will be issued after an order has been submitted.

EMMA conditions
Legally binding conditions for the transfer

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129P2/OlaHsd-Wt1tm1Mlh/H