Phenotypes Associated with This Genotype

Genotype
MGI:3720054

• Allelic Composition: Lefty2^tm2Hmd/Lefty2^tm2Hmd
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

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

mortality/aging

postnatal lethality, incomplete penetrance ( J:70483 )

• two-thirds of mice die within the first few days after birth due to cardiac defects associated with altred left-right patterning

• however, surviving mice have no cardiac defects

growth/size/body

left-sided isomerism ( J:70483 )

• however, lung lobe placement is normal

left atrial isomerism ( J:70483 )

• 6 of 50 mice exhibit atria left isomerism

cardiovascular system

abnormal hepatic portal vein morphology ( J:70483 )

• in 6 of 50 mice, the portal vein passes ventrally to the duodenum instead of dorsally as in wild-type mice

abnormal inferior vena cava morphology ( J:70483 )

• the left-sided inferior vena cava is connected to the azygos vein without its entering the liver as it does in wild-type mice

abnormal heart morphology ( J:70483 )

• cardiac abnormalities are associated with lethality and altered left-right patterning

abnormal heart and great artery attachment ( J:70483 )

• in 8 of 50 mice the pulmonary trunk and aorta are positioned side by side

• in 4 of 50 mice the aorta is ventral to the pulmonary artery

double outlet right ventricle ( J:70483 )

• in 3 of 8 mice

transposition of great arteries ( J:70483 )

• 2 of 8 mice exhibit a transposition of great arteries

tricuspid valve atresia ( J:70483 )

• in 1 of 8 mice

left atrial isomerism ( J:70483 )

• 6 of 50 mice exhibit atria left isomerism

dextrocardia ( J:70483 )

• in 3 of 50 mice

complete atrioventricular septal defect ( J:70483 )

• 1 in 8 mice exhibit a common atrioventricular canal

absent heart right ventricle ( J:70483 )

• in 1 of 8 mice

embryo

abnormal left-right axis patterning ( J:70483 )

• left-right patterning is distrupted with Pitx2 expression duplicated laterally and prolonged Nodal expression at the 8 somite stage

digestive/alimentary system

abnormal large intestine placement ( J:70483 )

• the colon fails to cross the duodenum

hematopoietic system

accessory spleen ( J:70483 )

• 8 of 50 mice exhibit one or more ectopic small spleens

liver/biliary system

abnormal liver morphology ( J:70483 )

• the right lateral and caudal lobes are fused in 8 of 50 mice and resemble the left lobe

• in 6 of 50 mice, the portal vein passes ventrally to the duodenum instead of dorsally as in wild-type mice

immune system

accessory spleen ( J:70483 )

• 8 of 50 mice exhibit one or more ectopic small spleens

respiratory system

respiratory system phenotype ( J:70483 )

N • despite left-right patterning defects in the heart, lung lobe placement is normal