Analysis Tools
craniofacial
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• entire enamel surface is roughened opaque and chalky white
• frequently have an irregular incisal edge
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• opaque and chalky white enamel
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• the upper and lower incisors are shortened
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• molars are opaque and roughened, rather than smooth and opalescent as in wild-type controls
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• molar teeth show abrasion of the cusps
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• at the ameloblast enamel interface the regular pattern of Tomes' process insertions is absent and instead there is a disorganized vesicular pattern
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• the upper and lower incisors are uneven and present a chalky white, opaque appearance with roughened/pitted surfaces
• large areas of the teeth lack enamel
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• in both incisors and molars, ameloblasts appear to lose contact with their sub-adjacent matrix and their cytoplasm becomes atypically eosinophilic
• in the transition and maturation zones cells become highly disorganized with the
ameloblasts losing their characteristic columnar morphology and forming a multi cellular
• much of debris of the multi cellular masses is either TUNEL- or activated caspase-3-positive
• eosinophilic structures are visible throughout the cell and appear to be vesicular in nature with accumulations of both amelogenin and ameloblastin in affected cells
• contact of the ameloblasts with the enamel matrix is lost leading to the formation of out-pocketings containing eosinophilic material
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• the mineralizing enamel distal to the white opaque zone is irregular and discolored with ridges perpendicular to the long axis of the incisor
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• by SEM enamel is severely dysplastic non-prismatic 'enamel' with a smooth glass-like appearance and no evidence of clear prismatic structure
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enamel pits
(
J:157947
)
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• the upper and lower incisors exhibit roughened/pitted surfaces
|
|
|
• molar teeth show abrasion of the cusps
|
|
|
• shortened with irregular incisal edge
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• mandible is enlarged, eroded and discolored, containing a large mass of soft tissue
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skeleton
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• entire enamel surface is roughened opaque and chalky white
• frequently have an irregular incisal edge
|
|
|
• opaque and chalky white enamel
|
|
|
• the upper and lower incisors are shortened
|
|
|
• molars are opaque and roughened, rather than smooth and opalescent as in wild-type controls
|
|
|
• molar teeth show abrasion of the cusps
|
|
|
• at the ameloblast enamel interface the regular pattern of Tomes' process insertions is absent and instead there is a disorganized vesicular pattern
|
|
|
• the upper and lower incisors are uneven and present a chalky white, opaque appearance with roughened/pitted surfaces
• large areas of the teeth lack enamel
|
|
|
• in both incisors and molars, ameloblasts appear to lose contact with their sub-adjacent matrix and their cytoplasm becomes atypically eosinophilic
• in the transition and maturation zones cells become highly disorganized with the
ameloblasts losing their characteristic columnar morphology and forming a multi cellular
• much of debris of the multi cellular masses is either TUNEL- or activated caspase-3-positive
• eosinophilic structures are visible throughout the cell and appear to be vesicular in nature with accumulations of both amelogenin and ameloblastin in affected cells
• contact of the ameloblasts with the enamel matrix is lost leading to the formation of out-pocketings containing eosinophilic material
|
|
|
• the mineralizing enamel distal to the white opaque zone is irregular and discolored with ridges perpendicular to the long axis of the incisor
|
|
|
• by SEM enamel is severely dysplastic non-prismatic 'enamel' with a smooth glass-like appearance and no evidence of clear prismatic structure
|
enamel pits
(
J:157947
)
|
|
• the upper and lower incisors exhibit roughened/pitted surfaces
|
|
|
• molar teeth show abrasion of the cusps
|
|
|
• shortened with irregular incisal edge
|
|
|
• mandible is enlarged, eroded and discolored, containing a large mass of soft tissue
|
growth/size/body
|
|
• entire enamel surface is roughened opaque and chalky white
• frequently have an irregular incisal edge
|
|
|
• opaque and chalky white enamel
|
|
|
• the upper and lower incisors are shortened
|
|
|
• molars are opaque and roughened, rather than smooth and opalescent as in wild-type controls
|
|
|
• molar teeth show abrasion of the cusps
|
|
|
• at the ameloblast enamel interface the regular pattern of Tomes' process insertions is absent and instead there is a disorganized vesicular pattern
|
|
|
• the upper and lower incisors are uneven and present a chalky white, opaque appearance with roughened/pitted surfaces
• large areas of the teeth lack enamel
|
|
|
• in both incisors and molars, ameloblasts appear to lose contact with their sub-adjacent matrix and their cytoplasm becomes atypically eosinophilic
• in the transition and maturation zones cells become highly disorganized with the
ameloblasts losing their characteristic columnar morphology and forming a multi cellular
• much of debris of the multi cellular masses is either TUNEL- or activated caspase-3-positive
• eosinophilic structures are visible throughout the cell and appear to be vesicular in nature with accumulations of both amelogenin and ameloblastin in affected cells
• contact of the ameloblasts with the enamel matrix is lost leading to the formation of out-pocketings containing eosinophilic material
|
|
|
• the mineralizing enamel distal to the white opaque zone is irregular and discolored with ridges perpendicular to the long axis of the incisor
|
|
|
• by SEM enamel is severely dysplastic non-prismatic 'enamel' with a smooth glass-like appearance and no evidence of clear prismatic structure
|
enamel pits
(
J:157947
)
|
|
• the upper and lower incisors exhibit roughened/pitted surfaces
|
|
|
• molar teeth show abrasion of the cusps
|
|
|
• shortened with irregular incisal edge
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amelogenesis imperfecta type 1E | DOID:0110058 |
OMIM:301200 |
J:157947 | |
