Analysis Tools
mortality/aging
|
• die soon after birth
|
craniofacial
|
• 3D microCT analysis of neonatal craniofacial bones showed alterations in the shape and volume of the premaxilla, maxilla, palatine bone, frontal bone, and mandible
• however, alterations in the half width and height of craniofacial bones are generally mild or undetectable
|
|
• the frontal suture is dramatically affected, as the gap between the paired neonatal frontal bones is increased by 1.3-fold relative to controls
|
|
• the width of the anterior narrow side of the neonatal frontal bone is increased by ~132%, whereas the width of the posterior wide side is reduced by only 12%
|
|
• the volume and surface area of the frontal bones are reduced by ~18% and 23%, respectively
|
|
• frontal bone length is reduced by ~11% relative to controls
• however, height is not significantly altered
|
|
• newborns show a significant loss of alveolar bone around both maxillary and mandibular incisors
|
|
• the length of neonatal maxillary and mandibular incisors is severely reduced
|
|
• the volume of the neonatal mandible is reduced by ~35% relative to controls
(J:264205)
|
|
• the volume of the neonatal mandible is reduced by ~35% relative to controls
(J:264205)
• however, the width is not significantly altered
(J:264205)
• mandibles show a 17% decrease in length, proportional to decrease in head size
(J:289243)
|
|
• the palatine process of the neonatal maxilla is severely disrupted, as the gap between the paired palatine processes of the maxilla is increased by ~5-fold relative to controls
|
|
• neonatal premaxillae show a 12-14% reduction in length and a 20% reduction in height, whereas width is increased by 6% relative to controls
• both the angle and extension of the posterior tip of the premaxilla are affected, altering its shape
|
|
• the volume of the premaxilla is reduced by 25% relative to controls
|
|
• neonatal maxillae exhibit a much smaller body, with a 18% reduction in length, a 25% reduction in half width and a 16% decrease in height relative to controls
• the volume of the whole maxilla is reduced by ~30% relative to controls
|
|
• neonatal palatine bones show a 35% reduction in length, whereas the gap between the paired bones is increased by 25-86%
• the volume of the palatine bones is reduced by ~30% relative to controls
• however, the half width and height of the palatine bones are relatively normal
|
|
• the horizontal palatine bones are almost completely absent, consistent with the cleft palate phenotype
|
|
• at E12.5, RNA expression of Msx1 is significantly reduced in the frontal bone primordium and the developing mandible
|
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• impaired proliferation of palatal mesenchymal cells
|
|
• delay in development at E14.5 but shelves have elevated at E15.5
|
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• at E15.5
|
|
• complete cleft palate with 100% penetrance
|
short tongue
(
J:289243
)
|
• proportional to decrease in head size
|
|
• show a 14.7% decrease in head length
|
behavior/neurological
skeleton
|
• 3D microCT analysis of neonatal craniofacial bones showed alterations in the shape and volume of the premaxilla, maxilla, palatine bone, frontal bone, and mandible
• however, alterations in the half width and height of craniofacial bones are generally mild or undetectable
|
|
• the frontal suture is dramatically affected, as the gap between the paired neonatal frontal bones is increased by 1.3-fold relative to controls
|
|
• the width of the anterior narrow side of the neonatal frontal bone is increased by ~132%, whereas the width of the posterior wide side is reduced by only 12%
|
|
• the volume and surface area of the frontal bones are reduced by ~18% and 23%, respectively
|
|
• frontal bone length is reduced by ~11% relative to controls
• however, height is not significantly altered
|
|
• newborns show a significant loss of alveolar bone around both maxillary and mandibular incisors
|
|
• the length of neonatal maxillary and mandibular incisors is severely reduced
|
|
• the volume of the neonatal mandible is reduced by ~35% relative to controls
(J:264205)
|
|
• the volume of the neonatal mandible is reduced by ~35% relative to controls
(J:264205)
• however, the width is not significantly altered
(J:264205)
• mandibles show a 17% decrease in length, proportional to decrease in head size
(J:289243)
|
|
• the palatine process of the neonatal maxilla is severely disrupted, as the gap between the paired palatine processes of the maxilla is increased by ~5-fold relative to controls
|
|
• neonatal premaxillae show a 12-14% reduction in length and a 20% reduction in height, whereas width is increased by 6% relative to controls
• both the angle and extension of the posterior tip of the premaxilla are affected, altering its shape
|
|
• the volume of the premaxilla is reduced by 25% relative to controls
|
|
• neonatal maxillae exhibit a much smaller body, with a 18% reduction in length, a 25% reduction in half width and a 16% decrease in height relative to controls
• the volume of the whole maxilla is reduced by ~30% relative to controls
|
|
• neonatal palatine bones show a 35% reduction in length, whereas the gap between the paired bones is increased by 25-86%
• the volume of the palatine bones is reduced by ~30% relative to controls
• however, the half width and height of the palatine bones are relatively normal
|
|
• the horizontal palatine bones are almost completely absent, consistent with the cleft palate phenotype
|
digestive/alimentary system
|
• the palatine process of the neonatal maxilla is severely disrupted, as the gap between the paired palatine processes of the maxilla is increased by ~5-fold relative to controls
|
|
• the horizontal palatine bones are almost completely absent, consistent with the cleft palate phenotype
|
|
• impaired proliferation of palatal mesenchymal cells
|
|
• delay in development at E14.5 but shelves have elevated at E15.5
|
|
• at E15.5
|
|
• complete cleft palate with 100% penetrance
|
short tongue
(
J:289243
)
|
• proportional to decrease in head size
|
growth/size/body
|
• newborns show a significant loss of alveolar bone around both maxillary and mandibular incisors
|
|
• the length of neonatal maxillary and mandibular incisors is severely reduced
|
|
• the palatine process of the neonatal maxilla is severely disrupted, as the gap between the paired palatine processes of the maxilla is increased by ~5-fold relative to controls
|
|
• the horizontal palatine bones are almost completely absent, consistent with the cleft palate phenotype
|
|
• impaired proliferation of palatal mesenchymal cells
|
|
• delay in development at E14.5 but shelves have elevated at E15.5
|
|
• at E15.5
|
|
• complete cleft palate with 100% penetrance
|
short tongue
(
J:289243
)
|
• proportional to decrease in head size
|
|
• show a 14.7% decrease in head length
|
microcephaly
(
J:289243
)
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| cleft palate | DOID:674 | J:289243 | ||
