Mouse Genome Informatics
hm1
    Amelxtm1Kul/Amelxtm1Kul
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

Enamel hypoplasia in Amelxtm1Kul/Amelxtm1Kul incisors

craniofacial
• teeth show a chalky-white discoloration as early as 2 weeks of age (J:71126)
• absence of prism pattern of organized mineral crystals in enamel (J:71126)
• mandibular molars show an enamel thickness with less than 10% of normal enamel (J:71126)
• tips of incisors and molars are broken (J:71126)

Mouse Models of Human Disease
OMIM IDRef(s)
Amelogenesis Imperfecta, Type IE; AI1E 301200 J:71126


Mouse Genome Informatics
cx2
    Amelxtm1Kul/Amelxtm1Kul
Tg(AMELX*P70T)2Gibs/?

involves: 129S1/Sv * 129X1/SvJ
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
tumorigenesis
• molar enamel has a hyperplastic intermedium cell layer exhibiting transition to tumor cells (J:122914)
• tumor is composed of benign, eosinophilic, polygonal-shaped cells with intercellular bridges (J:122914)
• calcification is not obvious in these tumors (J:122914)

craniofacial
• molar enamel has a hyperplastic intermedium cell layer exhibiting transition to tumor cells (J:122914)

Mouse Models of Human Disease
OMIM IDRef(s)
Amelogenesis Imperfecta, Type IE; AI1E 301200 J:122914


Mouse Genome Informatics
cx3
    Amelxtm1Kul/Amelxtm1Kul
Tg(AMELX-LRAP)9Gibs/0

involves: 129S1/Sv * 129X1/SvJ
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
craniofacial
• molar enamel surface has a rough appearance (J:122916)
• hypoplastic enamel lacks normal prism structure (J:122916)
• enamel thickness is 10-20 um in contrast to 100 um observed in wild-type mice (J:122916)