mortality/aging
|
• mice die shortly after birth
|
craniofacial
N |
• face development is grossly unaffected; neural crest-derived cells show normal distribution in all facial primordia at E10.5
|
|
• appearance of diastema teeth on the upper jaw
|
|
• a loss of molars is observed in the upper jaw
|
|
• lower molars are developmentally arrested
|
|
• at E13.5, a small localized decrease in mesenchymal cell proliferation is seen in the medial area of the anterior palate
• however, palatal mesenchyme shows no significant differences in apoptosis relative to controls
|
|
• starting at E13.5, the palatal shelf has an abnormal shape being more blunt and wider at the bottom; this is more pronounced in the anterior and middle levels than in the posterior level
• however, no adhesion between the palatal shelf and the tongue or oral epithelium is observed
|
|
• starting at E14.5, the palatal shelves are smaller in the anterior and posterior palate
|
|
• all mice exhibit fully cleft secondary palate at birth
|
|
• at E14.5, the middle palate and the posterior palate remain vertical, whereas the anterior palate is normally elevated above the tongue
• at E18.5, the posterior palate fails to elevate above the tongue, whereas the middle palate appears to have initiated re-orientation as shown by the presence of a protrusion on the medial side of the palatal shelf
• expression of Wnt5a, Pax9 and Osr2, which regulate palatal shelf elevation, is altered
• defect in middle/posterior palatal shelf elevation is not due to mechanical hindrance by the tongue
|
|
• at E14.5 and E18.5, the tongue is abnormally tall
• however, in vitro head culture assays show that the defect in middle/posterior palatal shelf elevation is not due to interference by the tongue
|
growth/size/body
|
• a loss of molars is observed in the upper jaw
|
|
• lower molars are developmentally arrested
|
|
• at E13.5, a small localized decrease in mesenchymal cell proliferation is seen in the medial area of the anterior palate
• however, palatal mesenchyme shows no significant differences in apoptosis relative to controls
|
|
• starting at E13.5, the palatal shelf has an abnormal shape being more blunt and wider at the bottom; this is more pronounced in the anterior and middle levels than in the posterior level
• however, no adhesion between the palatal shelf and the tongue or oral epithelium is observed
|
|
• starting at E14.5, the palatal shelves are smaller in the anterior and posterior palate
|
|
• all mice exhibit fully cleft secondary palate at birth
|
|
• at E14.5, the middle palate and the posterior palate remain vertical, whereas the anterior palate is normally elevated above the tongue
• at E18.5, the posterior palate fails to elevate above the tongue, whereas the middle palate appears to have initiated re-orientation as shown by the presence of a protrusion on the medial side of the palatal shelf
• expression of Wnt5a, Pax9 and Osr2, which regulate palatal shelf elevation, is altered
• defect in middle/posterior palatal shelf elevation is not due to mechanical hindrance by the tongue
|
|
• at E14.5 and E18.5, the tongue is abnormally tall
• however, in vitro head culture assays show that the defect in middle/posterior palatal shelf elevation is not due to interference by the tongue
|
digestive/alimentary system
|
• at E13.5, a small localized decrease in mesenchymal cell proliferation is seen in the medial area of the anterior palate
• however, palatal mesenchyme shows no significant differences in apoptosis relative to controls
|
|
• starting at E13.5, the palatal shelf has an abnormal shape being more blunt and wider at the bottom; this is more pronounced in the anterior and middle levels than in the posterior level
• however, no adhesion between the palatal shelf and the tongue or oral epithelium is observed
|
|
• starting at E14.5, the palatal shelves are smaller in the anterior and posterior palate
|
|
• all mice exhibit fully cleft secondary palate at birth
|
|
• at E14.5, the middle palate and the posterior palate remain vertical, whereas the anterior palate is normally elevated above the tongue
• at E18.5, the posterior palate fails to elevate above the tongue, whereas the middle palate appears to have initiated re-orientation as shown by the presence of a protrusion on the medial side of the palatal shelf
• expression of Wnt5a, Pax9 and Osr2, which regulate palatal shelf elevation, is altered
• defect in middle/posterior palatal shelf elevation is not due to mechanical hindrance by the tongue
|
|
• at E14.5 and E18.5, the tongue is abnormally tall
• however, in vitro head culture assays show that the defect in middle/posterior palatal shelf elevation is not due to interference by the tongue
|
skeleton
|
• appearance of diastema teeth on the upper jaw
|
|
• a loss of molars is observed in the upper jaw
|
|
• lower molars are developmentally arrested
|