All Phenotypes Wfdc2<tm1.1Ohbo> MGI Mouse

• lethality after birth, with no mice surviving post-weaning

• abnormal cilia morphology and shorter cilia in the P1.5 main bronchus

• less abundant cilia in the P1.5 main bronchus

lung inflammation ( J:282576 )

• inflammation in the neonatal lung

• mononuclear cells appear in alveolar spaces more often than in controls

abnormal lung morphology ( J:282576 )

• lungs show lower levels of phosphatidylcholine, but not phosphatidylgycerol

• abnormal lamellar body formation

atelectasis ( J:282576 )

• at P1.5, collapsed regions in the lungs are detected

• atelectasis is progressive

• 93.1% of mice that die between P0.5 and P1.5 have macroscopic atelectasis while 48.3% of the surviving mice have atelectasis

respiratory distress ( J:282576 )

• type II alveolar epithelial cells exhibit impairment of the processing of surfactants

cyanosis ( J:282576 )

• neonatal mice become cyanotic immediately after delivery and all die within 10 days

abnormal phospholipid level ( J:282576 )

• levels of phosphatidylcholine, but not phosphatidylgycerol, are lower in the lungs

abnormal cytokine level ( J:282576 )

• P1.5 mice show increased levels of pro-inflammatory and anti-inflammatory cytokines and various CC chemokines and CXC chemokines mRNAs

• 5.4-fold increase in IL-1alpha mRNA at P1.5

• 4.9-fold increase in TNF-beta mRNA at P1.5

• 11.5-fold and 16-fold increase in Ccl4/MIP1beta and Cxcl2/MIP-2 mRNA at P1.5