respiratory system
• mice exhibit reduced lung vascular branching compared to in wild-type mice
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• Background Sensitivity: abnormal lung development is more severe on an FVB background
• mice exhibit altered lung patterning and reduced lung vascular branching compared to in wild-type mice
• at E16.5, saccule expansion is delayed compared to in wild-type mice
• at E18.5, saccules are less opened than in wild-type mice
• in late embryogenesis, myofibroblast progenitors in the lungs exhibit defective differentiation marked by sma+ expression compared to in wild-type mice
• however, myofibroblast progenitor cell specification and spreading in the lungs are normal
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• mice exhibit thickened alveolar walls, lack of alveolar septation, and large alveolar size compared with wild-type mice
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skeleton
• rib patterning is abnormal
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limbs/digits/tail
short tail
(
J:155893
)
cardiovascular system
• mice exhibit reduced lung vascular branching compared to in wild-type mice
|