Phenotypes Associated with This Genotype

Genotype
MGI:3811312

• Allelic Composition: Bmpr1a^tm1Bhr/Bmpr1a^tm2.1Bhr; Tg(Sftpc-cre)1Blh/0
• Genetic Background: involves: 129S7/SvEvBrd * C57BL/6 * DBA/2 * ICR

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

mortality/aging

postnatal lethality, complete penetrance ( J:106392 )

• all mice die or are euthanized within 2 days of birth due to severe respiratory distress

respiratory system

respiratory system phenotype ( J:106392 )

N • despite defects in distal portions of the lungs, proximal bronchi and bronchioles appear normal

abnormal lung morphology ( J:106392 )

• at E16.5, the distal regions of all four lobes are abnormal

• lungs are highly abnormal with large, fluid-filled (emphysematous) sacs

• however, lungs are normal at E12.5

abnormal lung epithelium morphology ( J:106392 )

• at E16.5, the number of more distal branches is reduced and branches are smaller than in wild-type mice

• epithelial cells in the periphery of the lung have a more rounded morphology than in wild-type mice

• epithelial cells cultured in a mesenchyme-free system fail to undergo secondary branching, develop fewer or no buds and exhibit a collapsed and folded morphology compared to wild-type cultures

decreased type II pneumocyte number ( J:106392 )

• fewer than normal as determined by surfactant C expression

abnormal respiratory system physiology ( J:106392 )

• despite defects in distal portions of the lungs, proximal bronchi and bronchioles appear normal

respiratory distress ( J:106392 )