mortality/aging
• about 40% of mice display severe respiratory distress and die within 15 min of birth
• the remaining 60% of mice die within 24 h of birth
|
respiratory system
• impaired differentiation of type II pneumocytes
|
• decrease in saccular space at E18.5
• treatment with dexamethasone prior to birth restores saccular space but does not improve survival
|
• cells contain abundant glycogen granules and have attenuated villi indicating that the cells retain an immature phenotype
|
• at E18.5 lung weight is reduced by 25 - 40% and the lung weight to body weight ratio is significantly decreased
|
• at E18.5 the number of proliferating cells is reduced by 60% compared to wild-type littermates
• decreases in cell proliferation are significant by E16.5
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atelectasis
(
J:153218
)
• extensive and severe atelectasis is seen in mice that display respiratory distress
|
• severe distress is seen in about 40% of neonates
|
• about 40% of neonates die of acute respiratory failure within 15 min of birth
• lungs from these mice are not inflated with air
|
homeostasis/metabolism
behavior/neurological
• mice that survive more than 15 min. after birth display a reduced suckling reflex and lack milk in their stomachs
|
• mice that survive more than 15 min. after birth fail to respond to pinching
|
• mice that survive more than 15 min. after birth display uncoordinated movements
|
carpoptosis
(
J:153218
)
• seen in all mice
|
• mice that survive more than 15 min. after birth display infirm vocalization
|
skeleton
cardiovascular system
• at E18.5 heart weight is reduced by 25 - 40% and the heart weight to body weight ratio is significantly decreased
|
growth/size/body
• decrease in body weight compared to wild-type littermates is detected from E14.5 onwards
|
liver/biliary system
• at E18.5 liver weight is reduced by 25 - 40% and the liver weight to body weight ratio is significantly decreased
|