mortality/aging
|
• die between 2 and 3 weeks of age
|
respiratory system
|
• lungs have increased peripheral transparency and show surface bullae upon inflation, suggesting hyperinflation of the peripheral air spaces and attenuation of the parenchymal walls at the pleural surface
• the peripheral (subpleural) region of the lung is thinner
• isolated lung endothelial cells exhibit decreased transmigration through matrigel-coated fibers and decreased spontaneous formation of cord-like or tube-like structures
|
small lung
(
J:95881
)
|
• air spaces have smooth walls compared to the normal, irregularly corrugated walls of wild-type
• very few secondary septae are seen at 2 weeks of age, and when present, they are short and triangular in cross-section
|
|
• exhibit enlarged air saccules at 2 weeks of age with thinner walls between them
|
|
• by E16.5 and E18.5 but not earlier, show thinner distal bronchioles
|
endocrine/exocrine glands
|
• when grown in culture, submandibular gland rudiments exhibit fewer end buds and the end buds appear dilated, indicating defective branching morphogenesis
|
|
• submandibular glands at 2 weeks of age are smaller and have smaller lobules
|
digestive/alimentary system
|
• when grown in culture, submandibular gland rudiments exhibit fewer end buds and the end buds appear dilated, indicating defective branching morphogenesis
|
|
• submandibular glands at 2 weeks of age are smaller and have smaller lobules
|


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