Mouse Genome Informatics
hm1
    Ambntm1Nid/Ambntm1Nid
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype

Defects in enamel formation of Ambntm1Nid/Ambntm1Nid mice

tumorigenesis
• dental epithelium derived soft tissue tumors in the buccal vestibule of the maxilla were seen in 6 out of 32 mutants with the earliest occurrence at 26 weeks of age

craniofacial
• the space between the tooth and alveolar bone is wider than normal and this space is invaded by calcified tissue
• at P3 ameloblasts begin to detach from the matrix layer and lose cell polarity
• at P7 ameloblast cell polarity is completely lost, proliferation is increased, and the cells accumulate to form a multilayered structure
• at 3 weeks of age incisors appear chalky white with rounded tips
• the occlusal plane of the molar is flattened from attrition after eruption

skeleton
• the space between the tooth and alveolar bone is wider than normal and this space is invaded by calcified tissue

growth/size
• at P3 ameloblasts begin to detach from the matrix layer and lose cell polarity
• at P7 ameloblast cell polarity is completely lost, proliferation is increased, and the cells accumulate to form a multilayered structure
• at 3 weeks of age incisors appear chalky white with rounded tips
• the occlusal plane of the molar is flattened from attrition after eruption

Mouse Models of Human Disease
OMIM IDRef(s)
Amelogenesis Imperfecta, Hypoplastic Type 104530 J:94844