mortality/aging
• mutant newborns die within a day after birth
|
craniofacial
• at birth, mutant pups display flattened skulls
|
• mutant mandibular molars are fused with the oral ectoderm and the alveolar bone is absent
|
• at birth, mutant pups display small (only 5% of normal size) and abnormally shaped incisors in both the mandible and maxilla
|
• mandibular incisors display a single cusp with two symmetrical cervical loops; additional cusp formation is disrupted
|
• at birth, incisors are only 5% of normal size
|
• mandibular molars display a single irregular cusp; additional cusp formation is disrupted
|
small molars
(
J:65294
)
• at birth, mutant pups display small and abnormally shaped first molars in both the mandible and maxilla
• maxillary molars are less affected than mandibular molars which are 25% of normal size
• although cervical loops, dental papilla, inner enamel epithelium, predentin, and stellate reticulum are present, no dental cord is formed
|
• the dental cord is absent in mandibular molars
|
• at birth, mutant pups show absence of obvious teeth: manidbular molars and incisors exhibit a cap stage tooth rudiment of abnormal morphology
|
• at birth, mandibular incisors are more developmentally advanced relative to mandibular molars
|
• at birth, mandibular molars are less developmentally advanced relative to mandibular incisors
|
• in grafts of early tooth rudiments (13.5-15.5 dpc), dentin deposits are deposited but crown formation is incomplete and resulting teeth are small and abnormally shaped
|
• at birth, functional odontoblast layers are present but display abnormal polarity and cellular architecture
|
• at 14.5 dpc, the outer enamel epithelium of the lingual side is severely reduced and the lingual inner enamel epithelium has not invaginated, suggesting impaired crown formation
• when early tooth rudiments (13.5-15.5 dpc) are transplanted under kidney capsules of nude mice, enamel matrix is secreted but crown formation is incomplete and resulting teeth are small and abnormally shaped
|
• at birth, functional ameloblast layers are present but display abnormal polarity and cellular architecture
• when early tooth rudiments (13.5-15.5 dpc) are transplanted under kidney capsules of nude mice, enamel and dentin matrices are deposited in spite of absent ameloblast elongation and odontoblast disorganization
|
• at birth, mutant pups display a small frontal nasal process; nasal passageways are severely reduced
|
• the rudimentary palatal shelves are spaced widely apart
|
• the palatal shelves fail to develop beyond rudimentary processes
|
• 85% exhibit a cleft palate with rudimentary palatal shelves spaced widely apart
|
skeleton
N |
• at birth, mutant pups possess normal skeletal elements; the upper and lower jaws are of normal length
|
• at birth, mutant pups display flattened skulls
|
• mutant mandibular molars are fused with the oral ectoderm and the alveolar bone is absent
|
• at birth, mutant pups display small (only 5% of normal size) and abnormally shaped incisors in both the mandible and maxilla
|
• mandibular incisors display a single cusp with two symmetrical cervical loops; additional cusp formation is disrupted
|
• at birth, incisors are only 5% of normal size
|
• mandibular molars display a single irregular cusp; additional cusp formation is disrupted
|
small molars
(
J:65294
)
• at birth, mutant pups display small and abnormally shaped first molars in both the mandible and maxilla
• maxillary molars are less affected than mandibular molars which are 25% of normal size
• although cervical loops, dental papilla, inner enamel epithelium, predentin, and stellate reticulum are present, no dental cord is formed
|
• the dental cord is absent in mandibular molars
|
• at birth, mutant pups show absence of obvious teeth: manidbular molars and incisors exhibit a cap stage tooth rudiment of abnormal morphology
|
• at birth, mandibular incisors are more developmentally advanced relative to mandibular molars
|
• at birth, mandibular molars are less developmentally advanced relative to mandibular incisors
|
• in grafts of early tooth rudiments (13.5-15.5 dpc), dentin deposits are deposited but crown formation is incomplete and resulting teeth are small and abnormally shaped
|
• at birth, functional odontoblast layers are present but display abnormal polarity and cellular architecture
|
• at 14.5 dpc, the outer enamel epithelium of the lingual side is severely reduced and the lingual inner enamel epithelium has not invaginated, suggesting impaired crown formation
• when early tooth rudiments (13.5-15.5 dpc) are transplanted under kidney capsules of nude mice, enamel matrix is secreted but crown formation is incomplete and resulting teeth are small and abnormally shaped
|
• at birth, functional ameloblast layers are present but display abnormal polarity and cellular architecture
• when early tooth rudiments (13.5-15.5 dpc) are transplanted under kidney capsules of nude mice, enamel and dentin matrices are deposited in spite of absent ameloblast elongation and odontoblast disorganization
|
• at birth, mutant pups display a small frontal nasal process; nasal passageways are severely reduced
|
vision/eye
respiratory system
• at birth, mutant pups display a small frontal nasal process; nasal passageways are severely reduced
|
digestive/alimentary system
• the rudimentary palatal shelves are spaced widely apart
|
• the palatal shelves fail to develop beyond rudimentary processes
|
• 85% exhibit a cleft palate with rudimentary palatal shelves spaced widely apart
|
aerophagia
(
J:65294
)
• at birth, mutant pups are observed gulping air
|
integument
growth/size/body
• mutant mandibular molars are fused with the oral ectoderm and the alveolar bone is absent
|
• at birth, mutant pups display small (only 5% of normal size) and abnormally shaped incisors in both the mandible and maxilla
|
• mandibular incisors display a single cusp with two symmetrical cervical loops; additional cusp formation is disrupted
|
• at birth, incisors are only 5% of normal size
|
• mandibular molars display a single irregular cusp; additional cusp formation is disrupted
|
small molars
(
J:65294
)
• at birth, mutant pups display small and abnormally shaped first molars in both the mandible and maxilla
• maxillary molars are less affected than mandibular molars which are 25% of normal size
• although cervical loops, dental papilla, inner enamel epithelium, predentin, and stellate reticulum are present, no dental cord is formed
|
• the dental cord is absent in mandibular molars
|
• at birth, mutant pups show absence of obvious teeth: manidbular molars and incisors exhibit a cap stage tooth rudiment of abnormal morphology
|
• at birth, mandibular incisors are more developmentally advanced relative to mandibular molars
|
• at birth, mandibular molars are less developmentally advanced relative to mandibular incisors
|
• in grafts of early tooth rudiments (13.5-15.5 dpc), dentin deposits are deposited but crown formation is incomplete and resulting teeth are small and abnormally shaped
|
• at birth, functional odontoblast layers are present but display abnormal polarity and cellular architecture
|
• at 14.5 dpc, the outer enamel epithelium of the lingual side is severely reduced and the lingual inner enamel epithelium has not invaginated, suggesting impaired crown formation
• when early tooth rudiments (13.5-15.5 dpc) are transplanted under kidney capsules of nude mice, enamel matrix is secreted but crown formation is incomplete and resulting teeth are small and abnormally shaped
|
• at birth, functional ameloblast layers are present but display abnormal polarity and cellular architecture
• when early tooth rudiments (13.5-15.5 dpc) are transplanted under kidney capsules of nude mice, enamel and dentin matrices are deposited in spite of absent ameloblast elongation and odontoblast disorganization
|
• at birth, mutant pups display a small frontal nasal process; nasal passageways are severely reduced
|
• the rudimentary palatal shelves are spaced widely apart
|
• the palatal shelves fail to develop beyond rudimentary processes
|
• 85% exhibit a cleft palate with rudimentary palatal shelves spaced widely apart
|