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Phenotypes Associated with This Genotype
Genotype
MGI:5787924
Allelic
Composition
Alpltm1Jlm/Alpltm1Jlm
Genetic
Background
involves: 129S1/SvImJ * 129S2/SvPas * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Alpltm1Jlm mutation (0 available); any Alpl mutation (345 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
craniofacial
• 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

growth/size/body
• 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
• 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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
01/24/2023
MGI 6.22
The Jackson Laboratory