Analysis Tools
mortality/aging
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• no embryos are present at E15.5
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Allele Symbol Allele Name Allele ID |
Foxf1tm1Rhc targeted mutation 1, Robert H Costa MGI:2179135 |
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| Summary |
3 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• no embryos are present at E15.5
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• 5% of surviving pups die within 6 weeks of birth; the remaining 40% with near wild-type levels of Foxf1a expression have normal lifespans
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• 55% of newborns with lower than expected levels of Foxf1a expression die of severe lung hemorrhage within several hours of birth
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• at P0 but not E18 in mice that exhibit neonatal lethality, severe lung hemorrhage is seen
• red blood cells are detected in peripheral airspaces and in bronchioles
• lung hemorrhage is coincident with disruption of the mesenchymal-epithelial cell interfaces in the alveolar and bronchiolar regions
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• apoptotic cells are seen throughout the lung parenchyma, in smooth muscle cells underlying the bronchiolar epithelium, and in arterial smooth muscle cells in mice that exhibit pulmonary hemorrhage
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• impaired development of the alveolar capillaries is observed at birth
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• in mice that exhibit neonatal lethality, sacculation of the lung periphery is reduced indicating a failure to undergo differentiation of the terminal airspaces; a less severe defect in septation of the lung periphery is seen in surviving mice
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• reduced sacculation of the lung periphery in mice that exhibit neonatal lethality
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• neonates that die shortly after birth have breathing difficulties
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• reduced surfactant protein B (SP-B) expression in mice that exhibit neonatal lethality
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• impaired development of the alveolar capillaries is observed at birth
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• at P0 but not E18 in mice that exhibit neonatal lethality, severe lung hemorrhage is seen
• red blood cells are detected in peripheral airspaces and in bronchioles
• lung hemorrhage is coincident with disruption of the mesenchymal-epithelial cell interfaces in the alveolar and bronchiolar regions
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• neonatal lethality correlates with severe fusion of the right lung lobes and low pulmonary FoxF1a mRNA levels
• a mild fusion between the accessory and caudal lobes is insufficient to cause lethality
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• fusion of embryonic lung lobes is accompanied by misorientation and fusion of the large major pulmonary vessels
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• altered lung-bud orientation and reduced branching morphogenesis is noted at E10.5-E11; a caudal and lateral shift in the orientation of the accessory lobe, and a lateral shift in the middle and cranial lobe positions are commonly observed
• a range of fusion defects in the right pulmonary lobes are noted at E12-E13
• by E18, most embryonic lungs display fusion of the accessory and caudal lobes and abnormal orientation of the accessory lobe
• at E18, ~13% of embryonic lungs exhibit more severe malformations in which the cranial, caudal, and middle lobes are fused
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• at E11, impaired lung-bud formation is associated with reduced mesenchymal-epithelial interfaces
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• ~35-50% of heterozygous embryonic and newborn lungs exhibit severe lung fusions in the right lobes; however, only one severely fused lung is noted several days postnatally
• most adult heterozygotes display distinct cranial, middle, and caudal lobes and lack the severely fused lobes seen in embryos
• most adult heterozygotes show a mild fusion between the caudal and accessory right lobes resulting in a lateral shift in the position of the accessory lobe
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• fusion of embryonic lung lobes is accompanied by misorientation and fusion of the large major pulmonary vessels
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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 04/16/2024 MGI 6.23 |
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