mortality/aging
• mice die within 10 minutes of birth due to respiratory distress
|
respiratory system
• membrane blebs interrupts the arrangement of motile cilia in the lungs and membrane protrusions in the cell lining the bronchioles
|
• lung membrane blebs filled with proteinaceous material at E18.5
• membrane blebs interrupts the arrangement of motile cilia in the lungs and membrane protrusions in the cell lining the bronchioles
|
• blebs
|
• debris fills the airways with alveolar macrophages and eosinophilic fibrillar material
• decreased air space expansion
• focal proteinosis debris
• however, mice exhibit normal number of proximal airways and sacculations per section
|
• alveolar walls appear thickened and hypercellular
|
digestive/alimentary system
• gut lumen appears filled with debris perinatally unlike in wild-type mice
|
• rough topology
|
• perinatally
|
• fusion in the duodenum at E15.5 and E17.5
• fusion in the jejunum at E17.5
|
renal/urinary system
kidney cyst
(
J:206750
)
• at E18.5
|
• abnormal with partial loss in some tubules
|
cellular
• membrane blebs interrupts the arrangement of motile cilia in the lungs and membrane protrusions in the cell lining the bronchioles
|
growth/size/body
kidney cyst
(
J:206750
)
• at E18.5
|