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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Ergtm1.1Path
targeted mutation 1.1, Patricia A Thistlethwaite
MGI:5775397
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Ergtm1.1Path/Ergtm1.1Path Not Specified MGI:5775399


Genotype
MGI:5775399
hm1
Allelic
Composition
Ergtm1.1Path/Ergtm1.1Path
Genetic
Background
Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ergtm1.1Path mutation (0 available); any Erg mutation (27 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lethality between E16.5 and 3 months with 100% penetrance

cardiovascular system
• venous angiograms are abnormal with small-vessel pruning and absence of peripheral vascular blush
• however, mice show normal coronary artery/vein configuration and luminal diameter and coronary arteries and veins appear normal
• venule intimal thickening is correlated with cellular proliferation
• mice develop lethal pulmonary veno-occlusive disease, showing occlusion and luminal narrowing of postcapillary pulmonary venules
• pulmonary venule occlusion is widespread
• ratio of right ventricle weight to left ventricle and septum is increased in 4 week old mice
• variable pulmonary capillary hemorrhage
• postnatally, mice develop elevated right ventricular systolic pressures
• however, mice exhibit normal left ventricular systolic function, thickness, inner diameter, and fractional shortening
• pulmonary hypertension from venous obstruction in mice that survive past 4 weeks
• pulmonary venous endothelial cells (PVECs) show increased growth rates at perconfluence and increased proliferation
• however, pulmonary arterial endothelial cells (PAEC) growth rate and proliferation are normal

hematopoietic system

homeostasis/metabolism
• mice challenged with the pulmonary vasodilator epoprostenol develop life-threatening pulmonary edema

respiratory system
• variable pulmonary capillary hemorrhage
• mice challenged with the pulmonary vasodilator epoprostenol develop life-threatening pulmonary edema

cellular
• pulmonary venous endothelial cells (PVECs) show increased growth rates at perconfluence and increased proliferation
• however, pulmonary arterial endothelial cells (PAEC) growth rate and proliferation are normal

muscle
• ratio of right ventricle weight to left ventricle and septum is increased in 4 week old mice

growth/size/body
• ratio of right ventricle weight to left ventricle and septum is increased in 4 week old mice





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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory