Analysis Tools
growth/size/body
|
• surviving homozygotes show impaired growth
|
|
• surviving male and female mice show reduced body weights and slower weight gain from 5 weeks through adulthood
|
homeostasis/metabolism
integument
mortality/aging
|
• homozygotes surviving past 5 weeks have reduced lifespans, with greater than 50% mortality occurring between 20 and 50 weeks
|
|
• homozygotes are found at normal ratios throughout development, but frequency drops to 50% at postnatal day 1
• addition of the antioxidant n-acetyl-L-cysteine (NAC) to maternal drinking water to reduce oxidative stress on embryos prevents the observed neonatal mortality
|
respiratory system
|
• Clara cell markers are reduced in mutant lungs, suggesting that Clara cell differentiation could be affected
• cell proliferation is disrupted (reduced) at the saccular stage of lung development
• addition of the antioxidant n-acetyl-L-cysteine (NAC) to maternal drinking water to reduce oxidative stress on embryos normalizes lung morphology and prevents glycogen accumulation and ectopic CC10 expression
|
|
• saccules are poorly developed with thickened primary septae, increased glycogen deposits and widespread ectopic Clara cell marker CC10 expression at E19.5-P1; glycogen deposition and CC10 expression occur prior to birth
|
