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Phenotypes Associated with This Genotype
Genotype
MGI:5824267
Allelic
Composition
Wt1tm1.1Ndha/Wt1tm1.1Ndha
Tg(Gata4*G2-cre)#Roja/0
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Gata4*G2-cre)#Roja mutation (0 available)
Wt1tm1.1Ndha mutation (0 available); any Wt1 mutation (12 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• embryonic lethality around E15.5 (J:229936)
• fewer than the expected number of mutants are obtained at E15.5 (J:238024)

cardiovascular system
• embryos show a thin ventricular compact myocardium
• however, the epicardium remains intact
• impaired development of coronary vasculature
• embryos develop tortuous and sinusoidal irregular vessels that fail to progress transmurally and do not complete ventricular myocardium invasion
• normal coronary arteries are missing
• mutant N1ICD+ coronary endothelium does not form normal transmural coronary blood vessels, failing to connect to the deeper vascular elements of the developing coronary vasculature

embryo
• persistence of the intermediate mesoderm of the nephric ridges into the pleuroperitoneal folds
• fewer mesenchymal cells are present between the coelomic epithelium and the hepatoblasts at E10.5
• the mesenchymal cells of the posthepatic mesenchymal plate are located more medially, not in the lateral tips of the liver lobes, indicating that these cells cannot migrate towards the pleuroperitoneal folds
• some E12.5 embryos show strong reduction of the posthepatic mesenchymal plate size

muscle
• embryos show a thin ventricular compact myocardium
• however, the epicardium remains intact
• about 80% of E13.5 or older embryos show abnormal diaphragmatic development
• the closure of the pleural cavity by growth of the septum transversum/pleuroperitoneal folds crescent is delayed
• about 80% of mice develop Bochdaleks congenital diaphragmatic hernia, most frequently located in the left side
• diaphragmatic defect becomes apparent by E13.5, with 19 of 23 embryos showing diaphragmatic defects by E13.5 or older
• 3 of 23 embryos show thin, membranous diaphragm in the left side and 16 of 23 show a wide defect in the diaphragm, frequently with herniation of the liver into the left pleural cavity and hypoplasia of the left lung
• treatment of pregnant females with retinoic acid partially rescues diaphragm defects

respiratory system
• the closure of the pleural cavity by growth of the septum transversum/pleuroperitoneal folds crescent is delayed

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
congenital diaphragmatic hernia DOID:3827 OMIM:142340
OMIM:222400
OMIM:610187
J:238024


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
03/13/2019
MGI 6.13
The Jackson Laboratory