Analysis Tools
mortality/aging
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• mice die at birth as a result of a severe cleft palate defect
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craniofacial
| N |
• neural crest-derived cell migration, cell proliferation and apoptosis of palatal mesenchyme is unaffected
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• newborns exhibit multiple craniofacial malformations typical of Pierre Robin sequence in humans
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• severe defects with a significantly reduced size of craniofacial bones, including a ~40% reduction in zygomatic volume
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• Meckels cartilage is significantly smaller in size
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• smaller zygomatic process of squamous bone
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• maxillomandibular hypoplasia
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• coronoid process of the mandibular bone is missing
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• smaller and shorter jaw
• further analysis showed significant reduction in cell proliferation and differentiation of osteogenic progenitors in the mandible, contributing to micrognathia
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• ~10% reduction in mandibular bone length
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• smaller zygomatic process of the maxilla
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micrognathia
(
J:274818
)
|
• failed elevation of palatal shelves at E14.5
• palate shelves remain in the vertical position at E16.5
• however, no differences in cell proliferation or apoptosis are observed in the developing palatal shelves at E13.5
• in vitro, palatal shelves are able to elevate after 24 h in roller culture (when the mandible is removed) and to fuse after 72 h in organ culture, indicating that failed palatal shelf elevation is due to steric hindrance by the undescended tongue
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• newborns exhibit complete cleft palate due to failed palatal shelf elevation
|
|
• at E14.5, tongue position is significantly higher along the anterior-posterior axis
• however, tongue volume is not significantly altered at E13.5 and E14.5, and elevated tongue is not a consequence of increased cell proliferation or abnormal muscle patterning
|
skeleton
|
• severe defects with a significantly reduced size of craniofacial bones, including a ~40% reduction in zygomatic volume
|
|
• Meckels cartilage is significantly smaller in size
|
|
• smaller zygomatic process of squamous bone
|
|
• maxillomandibular hypoplasia
|
|
• coronoid process of the mandibular bone is missing
|
|
• smaller and shorter jaw
• further analysis showed significant reduction in cell proliferation and differentiation of osteogenic progenitors in the mandible, contributing to micrognathia
|
|
• ~10% reduction in mandibular bone length
|
|
• smaller zygomatic process of the maxilla
|
micrognathia
(
J:274818
)
digestive/alimentary system
|
• failed elevation of palatal shelves at E14.5
• palate shelves remain in the vertical position at E16.5
• however, no differences in cell proliferation or apoptosis are observed in the developing palatal shelves at E13.5
• in vitro, palatal shelves are able to elevate after 24 h in roller culture (when the mandible is removed) and to fuse after 72 h in organ culture, indicating that failed palatal shelf elevation is due to steric hindrance by the undescended tongue
|
|
• newborns exhibit complete cleft palate due to failed palatal shelf elevation
|
|
• at E14.5, tongue position is significantly higher along the anterior-posterior axis
• however, tongue volume is not significantly altered at E13.5 and E14.5, and elevated tongue is not a consequence of increased cell proliferation or abnormal muscle patterning
|
growth/size/body
|
• maxillomandibular hypoplasia
|
|
• coronoid process of the mandibular bone is missing
|
|
• smaller and shorter jaw
• further analysis showed significant reduction in cell proliferation and differentiation of osteogenic progenitors in the mandible, contributing to micrognathia
|
|
• ~10% reduction in mandibular bone length
|
|
• smaller zygomatic process of the maxilla
|
micrognathia
(
J:274818
)
|
• failed elevation of palatal shelves at E14.5
• palate shelves remain in the vertical position at E16.5
• however, no differences in cell proliferation or apoptosis are observed in the developing palatal shelves at E13.5
• in vitro, palatal shelves are able to elevate after 24 h in roller culture (when the mandible is removed) and to fuse after 72 h in organ culture, indicating that failed palatal shelf elevation is due to steric hindrance by the undescended tongue
|
|
• newborns exhibit complete cleft palate due to failed palatal shelf elevation
|
|
• at E14.5, tongue position is significantly higher along the anterior-posterior axis
• however, tongue volume is not significantly altered at E13.5 and E14.5, and elevated tongue is not a consequence of increased cell proliferation or abnormal muscle patterning
|
