- 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
129P2/OlaHsd-Wt1tm1Mlh/H
Status | Available to order |
EMMA ID | EM:04835 |
International strain name | 129P2/OlaHsd-Wt1tm1Mlh/H |
Alternative name | Wt1(tmT396) |
Strain type | Targeted Mutant Strains : Knock-in |
Allele/Transgene symbol | Wt1tm1Mlh, |
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 |
|
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
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)
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
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