mortality/aging
• die after birth
|
respiratory system
• late lung development is blocked such that lungs appear more cellular and contain immature alveoli and reduced airspace in distal lungs at E18.5 compared to wild-type
• marker analysis indicates that bronchiolar epithelial progenitor cell differentiation is impaired, however alveolar epithelial cell differentiation appears normal
|
• bronchiolar epithelial progenitor cell differentiation is impaired
|
• Clara cell numbers are reduced in E18.5 lungs
|
small lung
(
J:157922
)
• lungs are smaller at E15.5
|
• distal airways are composed of highly disorganized cells
|
• enlarged airways in the lungs at E15.5
|
integument
• fail to develop ventral skin
|