Phenotypes for Shh Yy1 MGI:5902322 MGI Mouse

neonatal lethality, complete penetrance ( J:239777 )

• all newborns die at birth from respiratory failure

lung cyst ( J:239777 )

• E18.5 lungs show the presence of dilated fluid-filled sacs

• proliferation assays in lungs from E18.5 fetuses show an increase in cell proliferation in cystic walls

• neither secretory club (Clara) cells or ciliated cells are seen in cyst epithelium at E18.5 and cysts are lined by Type II and Type I pneumocytes

• a microvascular network is present in the parenchyma forming the cyst walls

left pulmonary isomerism ( J:239777 )

• E12.5 lungs show two hypoplastic lobes instead of the expected asymmetric pattern of 4 right lobes and 1 left lobe

increased lung apoptosis ( J:239777 )

• apoptosis is increased in lung mesenchyme at E14.5

• E12.5 embryos cultured in vitro show increased apoptosis in the lung

• the addition of recombinant mouse SHH into the media rescues the increased apoptosis

abnormal lung morphology ( J:239777 )

• E18.5 lungs exhibit a disorganized architecture

lung cyst ( J:239777 )

• E18.5 lungs show the presence of dilated fluid-filled sacs

• proliferation assays in lungs from E18.5 fetuses show an increase in cell proliferation in cystic walls

• neither secretory club (Clara) cells or ciliated cells are seen in cyst epithelium at E18.5 and cysts are lined by Type II and Type I pneumocytes

• a microvascular network is present in the parenchyma forming the cyst walls

left pulmonary isomerism ( J:239777 )

• E12.5 lungs show two hypoplastic lobes instead of the expected asymmetric pattern of 4 right lobes and 1 left lobe

abnormal lung development ( J:239777 )

• marker analysis indicates altered patterning and cell differentiation in the lung

abnormal branching involved in lung morphogenesis ( J:239777 )

• branching morphogenesis in the lung is inhibited, however, distal epithelial cell differentiation is maintained

• E12.5 embryos cultured in vitro fail with branching of lung

• the addition of recombinant mouse SHH into the media does not rescue the branching defect

abnormal lung-associated mesenchyme development ( J:239777 )

• impairment of peribronchial smooth muscle differentiation as indicated by a lack of markers of airway smooth muscle differentiation around cysts

abnormal lung epithelium morphology ( J:239777 )

• lung epithelium exhibits an abnormal stratified structure at E12.5

decreased club cell number ( J:239777 )

• club (Clara) cells are scarce in the proximal airways

abnormal airway basal cell morphology ( J:239777 )

• basal cells are distributed irregularly along the proximal airways of embryos, although the number of basal cells is not altered

decreased respiratory mucosa goblet cell number ( J:239777 )

• goblet cells are scarce in the proximal airways

abnormal trachea morphology ( J:239777 )

• trachea is thinner with disorganized cartilage rings

• trachea is longer

abnormal tracheal ciliated epithelium morphology ( J:239777 )

• ciliated cells are scarce in the airways

abnormal tracheal cartilage morphology ( J:239777 )

• trachea shows abnormal formation of cartilage rings

trachea stenosis ( J:239777 )

respiratory failure ( J:239777 )

abnormal respiratory epithelium physiology ( J:239777 )

• reduction in lung epithelium proliferation at E12.5