Analysis Tools
craniofacial
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• severe dentinogenesis defects are associated with more extensive alveolar bone mineralization defects
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• when the entire root lacks dentin mineralization, pulp chambers are larger
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• reduction in dentinogenesis
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• disorganization of the odontoblast layer with flattened, dysmorphic odontoblasts lacking typical columnar morphology
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• root dentin of P14 mandibular molars shows developmental mineralization defects ranging in severity from mild to very severe, often within the same molar
• mild defects include a slight delay in mineralization of the predentin matrix to mineralized dentin matrix proper
• severe defects include complete lack of dentin mineralization and reduction in circumpulpal dentin formation, resulting in thin and unmineralized roots
• in the majority of mice, severe inhibition of dentin mineralization is on the lingual aspect of the first molar while the contralateral buccal side is mildly affected
• by P21, the mildly affected buccal dentin of molars is well mineralized, indicating that delayed mineralization of predentin at P14 is corrected by P21
• mice treated with a human from of TNAP, ENB-0040, a form of enzyme replacement therapy, exhibit normal molar root dentin formation and mineralization, however dentin of the incisor root is not rescued
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• by P21, the root dentin mineralization of the lingual aspect of molars remains arrested, resulting in a root that continues to grow in length but does not advance dentin apposition and an atypical tissue layer forms outside the thin and unmineralized roots, resembling unmineralized cementum
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• when the entire root lacks dentin mineralization, root lengths are shorter and root shape is dysmorphic
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• poorly mineralized tooth molar and incisor roots
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growth/size/body
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• severe dentinogenesis defects are associated with more extensive alveolar bone mineralization defects
|
|
• when the entire root lacks dentin mineralization, pulp chambers are larger
|
|
• reduction in dentinogenesis
|
|
• disorganization of the odontoblast layer with flattened, dysmorphic odontoblasts lacking typical columnar morphology
|
|
• root dentin of P14 mandibular molars shows developmental mineralization defects ranging in severity from mild to very severe, often within the same molar
• mild defects include a slight delay in mineralization of the predentin matrix to mineralized dentin matrix proper
• severe defects include complete lack of dentin mineralization and reduction in circumpulpal dentin formation, resulting in thin and unmineralized roots
• in the majority of mice, severe inhibition of dentin mineralization is on the lingual aspect of the first molar while the contralateral buccal side is mildly affected
• by P21, the mildly affected buccal dentin of molars is well mineralized, indicating that delayed mineralization of predentin at P14 is corrected by P21
• mice treated with a human from of TNAP, ENB-0040, a form of enzyme replacement therapy, exhibit normal molar root dentin formation and mineralization, however dentin of the incisor root is not rescued
|
|
• by P21, the root dentin mineralization of the lingual aspect of molars remains arrested, resulting in a root that continues to grow in length but does not advance dentin apposition and an atypical tissue layer forms outside the thin and unmineralized roots, resembling unmineralized cementum
|
|
• when the entire root lacks dentin mineralization, root lengths are shorter and root shape is dysmorphic
|
|
• poorly mineralized tooth molar and incisor roots
|
skeleton
|
• severe dentinogenesis defects are associated with more extensive alveolar bone mineralization defects
|
|
• when the entire root lacks dentin mineralization, pulp chambers are larger
|
|
• reduction in dentinogenesis
|
|
• disorganization of the odontoblast layer with flattened, dysmorphic odontoblasts lacking typical columnar morphology
|
|
• root dentin of P14 mandibular molars shows developmental mineralization defects ranging in severity from mild to very severe, often within the same molar
• mild defects include a slight delay in mineralization of the predentin matrix to mineralized dentin matrix proper
• severe defects include complete lack of dentin mineralization and reduction in circumpulpal dentin formation, resulting in thin and unmineralized roots
• in the majority of mice, severe inhibition of dentin mineralization is on the lingual aspect of the first molar while the contralateral buccal side is mildly affected
• by P21, the mildly affected buccal dentin of molars is well mineralized, indicating that delayed mineralization of predentin at P14 is corrected by P21
• mice treated with a human from of TNAP, ENB-0040, a form of enzyme replacement therapy, exhibit normal molar root dentin formation and mineralization, however dentin of the incisor root is not rescued
|
|
• by P21, the root dentin mineralization of the lingual aspect of molars remains arrested, resulting in a root that continues to grow in length but does not advance dentin apposition and an atypical tissue layer forms outside the thin and unmineralized roots, resembling unmineralized cementum
|
|
• when the entire root lacks dentin mineralization, root lengths are shorter and root shape is dysmorphic
|
|
• poorly mineralized tooth molar and incisor roots
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• loss of bone mineralization in the mandible
• severe dentinogenesis defects are associated with more extensive alveolar bone mineralization defects
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| infantile hypophosphatasia | DOID:0110914 |
OMIM:241500 |
J:233260 | |
