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Phenotypes Associated with This Genotype
Genotype
MGI:6470789
Allelic
Composition
Foxf1em1Vvk/Foxf1+
Genetic
Background
involves: C57BL/6 * DBA/2
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Foxf1em1Vvk mutation (0 available); any Foxf1 mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 72% mortality rate by 1 month of age

respiratory system
• embryos show loss of microvascular network in the peripheral lung
• newborn lungs show hemorrhage
• accumulation of fibrin in alveolar regions and pulmonary bronchioles consistent with persistent vascular leak and hemorrhage
• nanoparticle-mediated delivery of STAT3 to newborns decreases lung inflammation
• proliferation of endothelial cells is reduced in the lungs
• nanoparticle-mediated delivery of STAT3 to newborns (into the facial vein) restores pulmonary endothelial proliferation
• newborn lungs show pulmonary inflammation
• lungs of newborn mice exhibit fusion of the right lobes
• lungs of newborn mice are smaller
• surviving mice exhibit alveolar simplification
• nanoparticle-mediated delivery of STAT3 to newborns improves alveogenesis

cardiovascular system
• newborn lungs show hypertrophy of pulmonary arteries
• embryos show loss of microvascular network in the peripheral lung
• newborns show reduced lung angiogenesis
• nanoparticle-mediated delivery of STAT3 to newborns stimulates angiogenesis in the lungs
• newborns and embryos show multiple veins close to airways and arteries, a feature of misalignment of pulmonary veins seen in patients with alveolar capillary dysplasia with misalignment of pulmonary veins
• newborn lungs show hemorrhage
• accumulation of fibrin in alveolar regions and pulmonary bronchioles consistent with persistent vascular leak and hemorrhage
• nanoparticle-mediated delivery of STAT3 to newborns decreases lung inflammation

cellular
• proliferation of endothelial cells is reduced in the lungs
• nanoparticle-mediated delivery of STAT3 to newborns (into the facial vein) restores pulmonary endothelial proliferation

endocrine/exocrine glands
• gallbladders are either absent or underdeveloped
• however, no histologic abnormalities in the trachea, heart, kidney, and intestine are seen
• in some mice

growth/size/body
• progressive decrease of body weight during the postnatal period

immune system
• newborn lungs show pulmonary inflammation

liver/biliary system
• gallbladders are either absent or underdeveloped
• however, no histologic abnormalities in the trachea, heart, kidney, and intestine are seen
• in some mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
persistent fetal circulation syndrome DOID:13042 OMIM:265380
J:296328


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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last database update
05/10/2022
MGI 6.19
The Jackson Laboratory