Phenotypes for Foxf1 MGI:3613577 MGI Mouse

premature death ( J:70364 )

• 5% of surviving pups die within 6 weeks of birth; the remaining 40% with near wild-type levels of Foxf1a expression have normal lifespans

neonatal lethality, incomplete penetrance ( J:70364 )

• 55% of newborns with lower than expected levels of Foxf1a expression die of severe lung hemorrhage within several hours of birth

lung hemorrhage ( J:70364 )

• 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

abnormal lung morphology ( J:70364 )

• 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

abnormal lung vasculature morphology ( J:70364 )

• impaired development of the alveolar capillaries is observed at birth

impaired lung alveolus development ( J:70364 )

• 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

abnormal lung saccule morphology ( J:70364 )

• reduced sacculation of the lung periphery in mice that exhibit neonatal lethality

respiratory distress ( J:70364 )

• neonates that die shortly after birth have breathing difficulties

abnormal surfactant secretion ( J:70364 )

• reduced surfactant protein B (SP-B) expression in mice that exhibit neonatal lethality