Phenotypes Associated with This Genotype

Genotype
MGI:3793499

• Allelic Composition: Wnt7b^tm2Amc/Wnt7b^tm2.1Amc; Edil3^{Tg(Sox2-cre)1Amc}/Edil3⁺
• Genetic Background: involves: 129X1/SvJ * C57BL/6 * CBA

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

mortality/aging

neonatal lethality, complete penetrance ( J:134483 )

• mice die within hours of birth

respiratory system

lung hemorrhage ( J:134483 )

abnormal lung development ( J:134483 )

• lung epithelial and mesenchyme tip cell progenitors exhibit decreased replication compared to in wild-type mice

• however, normal cell differentiation and patterning are preserved

decreased mesenchymal cell proliferation involved in lung development ( J:134483 )

• mesenchyme tip cell progenitors exhibit decreased replication compared to in wild-type mice

thin lung-associated mesenchyme ( J:134483 )

• the lung mesenchyme is thinner than in wild-type mice

abnormal right lung middle lobe morphology ( J:134483 )

• the medial lobe bronchus of the right lung emanates from the cephalic lobe bronchus, unlike in wild-type mice

• however, the correct number of lobes is formed

small lung ( J:134483 )

• at E12.5

decreased lung weight ( J:134483 )

• lung weight is 50% of wild-type at E18.5 and 25% of wild-type at E14.5

atelectasis ( J:134483 )

• lungs are poorly inflated

abnormal tracheal cartilage morphology ( J:134483 )

• cartilaginous rings are incomplete

homeostasis/metabolism

cyanosis ( J:134483 )

• after birth mice become cyanotic despite chest wall movements

renal/urinary system

increased kidney apoptosis ( J:142685 )

• at E17.5, a marked increase in the rate of apoptosis is seen in distal collecting duct epithelial cells

abnormal kidney cell proliferation ( J:142685 )

• at E15.5, the majority of cells in the collecting duct divide along the radial axis unlike in controls where the majority of cells divide along the longitudinal axis

decreased kidney cell proliferation ( J:142685 )

• cell proliferation in the loop of Henle is significantly reduced

abnormal kidney morphology ( J:142685 )

• renal corpuscles abut the renal pelvis

• despite the absence of the medulla, kidneys are similar in size to controls

abnormal kidney collecting duct epithelium morphology ( J:142685 )

• at E15.5, the majority of cells in the collecting duct divide along the radial axis unlike in controls where the majority of cells divide along the longitudinal axis

• at E17.5, a marked increase in the rate of apoptosis is seen in distal collecting duct epithelial cells

abnormal kidney medulla development ( J:142685 )

• at E15.5 and E16.5, the nascent medulla is absent indicating a failure to initiate medulla formation

absent kidney medulla ( J:142685 )

• absent at E18.5

truncated loop of Henle ( J:142685 )

• at E18.5 the loop of Henle is truncated resembling morphology at an earlier stage prior to loop elongation

• cell proliferation in the loop of Henle is significantly reduced

hydroureter ( J:142685 )

• at E18.5, less than a third of mice show hydroureter like swelling of the pelvic region

skeleton

abnormal tracheal cartilage morphology ( J:134483 )

• cartilaginous rings are incomplete

cardiovascular system

lung hemorrhage ( J:134483 )

cellular

increased kidney apoptosis ( J:142685 )

• at E17.5, a marked increase in the rate of apoptosis is seen in distal collecting duct epithelial cells

abnormal kidney cell proliferation ( J:142685 )

• at E15.5, the majority of cells in the collecting duct divide along the radial axis unlike in controls where the majority of cells divide along the longitudinal axis

decreased kidney cell proliferation ( J:142685 )

• cell proliferation in the loop of Henle is significantly reduced

decreased mesenchymal cell proliferation involved in lung development ( J:134483 )

• mesenchyme tip cell progenitors exhibit decreased replication compared to in wild-type mice