mortality/aging
• homozygotes die around E17.5, with no live mice found at E18.5 or P0.5
|
growth/size/body
• slightly smaller than heterozygotes at E17.5
|
• slightly smaller than heterozygotes at E13.5 and E17.5
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respiratory system
• maturation of lamellar bodies in type II pneumocytes is impaired, as shown by the presence of small and lipid-less lamellar bodies at E17.5
|
• at E17.5, lamellar bodies labeled with surfactant protein-C (SPC) are smaller than those in heterozygous control mice
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small lung
(
J:300057
)
• small lung size at E17.0
|
homeostasis/metabolism
• analysis of lipid content in whole-lung homogenates from E17.0 embryos revealed normal levels of phospholipids (phosphatidyl-choline (DPPC), phosphatidylethanolamine, phosphatidyl-glycerol and phosphatidylinositol), suggesting that delivery of phospholipids from the ER to lamellar bodies, but not synthesis, is impaired
|