Mouse Genome Informatics
hm
    Zfpm2lil/Zfpm2lil
involves: A/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Abnormal pulmonary and diaphragmatic development in the Zfpm2lil/Zfpm2lil mouse

mortality/aging
• progressive loss of embryos is seen from E13.5 to E15.5; number surviving to birth is less than 5% of expected value
• 1 viable embryo was found on E18.5, while some mice were found dead after birth

respiratory system
• underdevelopment of the anterior portion of the right lung middle lobe
• bilateral pulmonary hypoplasia including absence of an accessory lobe on the right side and underdevelopment of the anterior right middle lobe is seen

cardiovascular system
• at E15.5 the endocardial cushions are enlarged and abnormally developed
• decreased vascularization of the myocardium is found at E15.5
• ostium primum atrial septal defects are seen in homozygotes that survive to birth
• enlarged atria are seen in homozygotes that survive to birth
• thinning of the outer compact layer at E15.5
• at E15.5, the myocardium is poorly developed with thinning of the outer compact layer
• at E15.5 some embryos still have a complete atrioventricular canal
• atrioventricular canal type ventricular septal defects are seen in homozygotes that survive to birth

muscle
• the diaphragm is intact but muscularization is absent in the dorsal regions with the few myotubes that are present radiating in a dorsal-ventral orientation and muscletissue does not meet the entire surface of the body walls

Mouse Models of Human Disease
OMIM IDRef(s)
Diaphragmatic Hernia, Congenital 142340 J:100119