Analysis Tools
craniofacial
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• all craniofacial bones are thinner compared to wild-type controls
• density of craniofacial bones is reduced as early as E18.5
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• enlarged intermaxillary spaces or extended sutures at 9 weeks of age
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• higher cranial vault
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• more rounded skull with a higher cranial vault
• skull centroid sizes become more similar to wild-type controls with age suggesting a delay in ontogenetic development
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• relatively longer, appearing to compensate for decreased length of the nasal and supraoccipital bones
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• relatively longer, appearing to compensate for decreased length of the nasal and supraoccipital bones
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• relatively shorter compared to wild-type controls
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• lack the opening normally seen in the temporal bone
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• decrease in the length of individual bones in the skull vault
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• rather than regressing as in wild-type mice, diastema tooth buds survive
• thinning is especially evident in suture forming areas
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• an ectopic tooth is found rostrally of the first molar in the mandibular diastema region or the first molar is expanded with a rostral ectopic cusp
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• on the anterior end of M1, the cusps are farther apart resulting in shortening of M1
• however, other molar cups are essentially normal, unlike in other mice with supernumerary teeth
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• rather than regressing as in wild-type mice, diastema tooth buds survive
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• in the mandible 92% of mice have bilateral diastema teeth, 5% unilateral and 3% no diastema teeth
(J:119280)
• less than 5% of mice have supernumerary teeth in the maxilla
(J:119280)
• rudimentary tooth bud is present at E14.5 in the diastemal area in the mandible
(J:315670)
|
malocclusion
(
J:315670
)
|
• mandibular and maxillary molars do not properly occlude
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• abnormally shaped mandibular notches
• mandibles tend to have longer toothrow, longer and backward shifted processus coronoideus, slightly shorter incisors, and shortened angular process compared to wild-type controls
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• abnormally shaped
• irregular articular cartilage surfaces throughout the condyle
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• abnormally shaped
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• vomeronasal bones display posterior curvature that prevents formation of the vomeromaxillary suture within the palatine process of the maxilla
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• relatively shorter nasal bone compared to wild-type controls
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cleft palate
(
J:315670
)
|
• complete and incomplete cleft palates are seen
|
short snout
(
J:315670
)
cellular
|
• axoneme elongation is seen in postnatal but not prenatal tibial and mandibular condyle chondrocytes
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digestive/alimentary system
|
• vomeronasal bones display posterior curvature that prevents formation of the vomeromaxillary suture within the palatine process of the maxilla
|
cleft palate
(
J:315670
)
|
• complete and incomplete cleft palates are seen
|
skeleton
|
• all craniofacial bones are thinner compared to wild-type controls
• density of craniofacial bones is reduced as early as E18.5
|
|
• enlarged intermaxillary spaces or extended sutures at 9 weeks of age
|
|
• higher cranial vault
|
|
• more rounded skull with a higher cranial vault
• skull centroid sizes become more similar to wild-type controls with age suggesting a delay in ontogenetic development
|
|
• relatively longer, appearing to compensate for decreased length of the nasal and supraoccipital bones
|
|
• relatively longer, appearing to compensate for decreased length of the nasal and supraoccipital bones
|
|
• relatively shorter compared to wild-type controls
|
|
• lack the opening normally seen in the temporal bone
|
|
• decrease in the length of individual bones in the skull vault
|
|
• rather than regressing as in wild-type mice, diastema tooth buds survive
• thinning is especially evident in suture forming areas
|
|
• an ectopic tooth is found rostrally of the first molar in the mandibular diastema region or the first molar is expanded with a rostral ectopic cusp
|
|
• on the anterior end of M1, the cusps are farther apart resulting in shortening of M1
• however, other molar cups are essentially normal, unlike in other mice with supernumerary teeth
|
|
• rather than regressing as in wild-type mice, diastema tooth buds survive
|
|
• in the mandible 92% of mice have bilateral diastema teeth, 5% unilateral and 3% no diastema teeth
(J:119280)
• less than 5% of mice have supernumerary teeth in the maxilla
(J:119280)
• rudimentary tooth bud is present at E14.5 in the diastemal area in the mandible
(J:315670)
|
malocclusion
(
J:315670
)
|
• mandibular and maxillary molars do not properly occlude
|
|
• abnormally shaped mandibular notches
• mandibles tend to have longer toothrow, longer and backward shifted processus coronoideus, slightly shorter incisors, and shortened angular process compared to wild-type controls
|
|
• abnormally shaped
• irregular articular cartilage surfaces throughout the condyle
|
|
• abnormally shaped
|
|
• vomeronasal bones display posterior curvature that prevents formation of the vomeromaxillary suture within the palatine process of the maxilla
|
|
• relatively shorter nasal bone compared to wild-type controls
|
|
• in the tibia the growth plate is extended compared to wild-type controls
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• reduced in size
• bone thickness is reduced in all areas along the vertebral column
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• reduced size is especially noticeable in the vertebral processes and most pronounced in the processus spinosus
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• the transverse process of the atlas/axis is not fully formed and the transverse foramen is not closed at the caudal aspect in some cases
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• reduced in size
• bone thickness is reduced in all areas of the vertebral column
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• spinal stenosis
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• density of craniofacial bones is reduced as early as E18.5
|
|
• axoneme elongation is seen in primary cilia of postnatal but not prenatal tibial and mandibular condyle chondrocytes
|
growth/size/body
|
• an ectopic tooth is found rostrally of the first molar in the mandibular diastema region or the first molar is expanded with a rostral ectopic cusp
|
|
• on the anterior end of M1, the cusps are farther apart resulting in shortening of M1
• however, other molar cups are essentially normal, unlike in other mice with supernumerary teeth
|
|
• rather than regressing as in wild-type mice, diastema tooth buds survive
|
|
• in the mandible 92% of mice have bilateral diastema teeth, 5% unilateral and 3% no diastema teeth
(J:119280)
• less than 5% of mice have supernumerary teeth in the maxilla
(J:119280)
• rudimentary tooth bud is present at E14.5 in the diastemal area in the mandible
(J:315670)
|
malocclusion
(
J:315670
)
|
• mandibular and maxillary molars do not properly occlude
|
|
• vomeronasal bones display posterior curvature that prevents formation of the vomeromaxillary suture within the palatine process of the maxilla
|
|
• relatively shorter nasal bone compared to wild-type controls
|
cleft palate
(
J:315670
)
|
• complete and incomplete cleft palates are seen
|
short snout
(
J:315670
)
respiratory system
|
• relatively shorter nasal bone compared to wild-type controls
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| ciliopathy | DOID:0060340 | J:315670 | ||
