Phenotypes associated with this allele
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
AmelxRgsc888 mutation
(0 available);
any
Amelx mutation
(25 available)
|
|
|
craniofacial
|
• severely hypoplastic
• large areas of enamel are missing
|
|
• in incisors ameloblasts appear to lose contact with their subadjacent 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 mass
|
growth/size/body
|
• severely hypoplastic
• large areas of enamel are missing
|
|
• in incisors ameloblasts appear to lose contact with their subadjacent 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 mass
|
skeleton
|
• severely hypoplastic
• large areas of enamel are missing
|
|
• in incisors ameloblasts appear to lose contact with their subadjacent 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 mass
|
Allelic Composition |
AmelxRgsc888/Amelx+
|
|
Genetic Background |
involves: C57BL/6JJcl * DBA/2J |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
AmelxRgsc888 mutation
(0 available);
any
Amelx mutation
(25 available)
|
|
|
craniofacial
|
• incisors display patchy regions of roughened, chalky white enamel
|
|
• patchy regions of chalky white enamel
|
|
• the upper and lower incisors are shortened
|
|
• incisors display patchy regions of roughened, chalky white enamel
|
|
• occasional small blister like structures of variable size are apparent from the late secretory stage onwards
• within the blister like structures aberrant matrix is present with areas appearing less eosinophilic than the original enamel matrix
|
|
• decrease in enamel mineral content
• no defect in dentine mineral levels
|
|
• by SEM the prismatic structure is less highly ordered with greater inter-prismatic spacing
|
|
• shortened with irregular incisal edge
|
skeleton
|
• incisors display patchy regions of roughened, chalky white enamel
|
|
• patchy regions of chalky white enamel
|
|
• the upper and lower incisors are shortened
|
|
• incisors display patchy regions of roughened, chalky white enamel
|
|
• occasional small blister like structures of variable size are apparent from the late secretory stage onwards
• within the blister like structures aberrant matrix is present with areas appearing less eosinophilic than the original enamel matrix
|
|
• decrease in enamel mineral content
• no defect in dentine mineral levels
|
|
• by SEM the prismatic structure is less highly ordered with greater inter-prismatic spacing
|
|
• shortened with irregular incisal edge
|
growth/size/body
|
• incisors display patchy regions of roughened, chalky white enamel
|
|
• patchy regions of chalky white enamel
|
|
• the upper and lower incisors are shortened
|
|
• incisors display patchy regions of roughened, chalky white enamel
|
|
• occasional small blister like structures of variable size are apparent from the late secretory stage onwards
• within the blister like structures aberrant matrix is present with areas appearing less eosinophilic than the original enamel matrix
|
|
• decrease in enamel mineral content
• no defect in dentine mineral levels
|
|
• by SEM the prismatic structure is less highly ordered with greater inter-prismatic spacing
|
|
• shortened with irregular incisal edge
|
Allelic Composition |
AmelxRgsc888/Amelx+
|
|
Genetic Background |
involves: C57BL/6JJcl * DBA/2JJcl |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
AmelxRgsc888 mutation
(0 available);
any
Amelx mutation
(25 available)
|
|
|
craniofacial
growth/size/body
skeleton
Allelic Composition |
AmelxRgsc888/Y
|
|
Genetic Background |
involves: C57BL/6JJcl * DBA/2J |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
AmelxRgsc888 mutation
(0 available);
any
Amelx mutation
(25 available)
|
|
|
craniofacial
|
• 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
|
|
• 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
|
skeleton
|
• 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
|
|
• 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
|
|
• the upper and lower incisors exhibit roughened/pitted surfaces
|
|
• molar teeth show abrasion of the cusps
|
|
• shortened with irregular incisal edge
|