Phenotypes Associated with This Genotype

Genotype
MGI:3575016

• Allelic Composition: Mycn^tm1Psk/Mycn⁺; Tg(Sftpc-cre)1Blh/0
• Genetic Background: involves: 129 * C57BL/6 * ICR

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

decreased survivor rate ( J:97215 )

• ~50% of mutants develop normally into adulthood with no obvious lung defects

neonatal lethality, incomplete penetrance ( J:97215 )

• ~50% of mutants die at or shortly after birth with a severe lung phenotype

respiratory system

abnormal lung morphology ( J:97215 )

• at E18.5 the lungs of affected mutants are composed of numerous large fluid-filled sacs containing cellular debris lined by highly attenuated epithelial cells separated by a thin layer of mesoderm

• however, lung size, lobulation pattern, and trachea morphology are normal

abnormal lung development ( J:97215 )

• at E15.5-E16.5, a reduction in the proportion of proliferating cells in the endoderm is seen

• at E15.5, E16.5 and E18.5, abundant apoptotic cells are detected in the lumen, epithelium and mesenchyme, unlike in wild-type lungs

abnormal branching involved in lung morphogenesis ( J:97215 )

• at E14.5-E16.5 in affected mutants, branching is reduced with only a few expanded tubes separated by abundant mesoderm present

abnormal lung epithelium morphology ( J:97215 )

• at E14.5, E15.5 and E18.5, epithelial cell size is irregular, dead cells are seen in the lumen, and increased apoptosis is seen

decreased type I pneumocyte number ( J:97215 )

• at E18.5, attenuated presumptive type I cells are lining the large sacs

decreased type II pneumocyte number ( J:97215 )

• at E18.5, a paucity of type II cells is observed in the mutant lung