Mouse Genome Informatics
hm1
    Amelxtm1Kul/Amelxtm1Kul
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype

Enamel hypoplasia in Amelxtm1Kul/Amelxtm1Kul incisors

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

growth/size/body
• teeth show a chalky-white discoloration as early as 2 weeks of age
• absence of prism pattern of organized mineral crystals in enamel
• mandibular molars show an enamel thickness with less than 10% of normal enamel
• tips of incisors and molars are broken

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
phenotype observed in males
N normal phenotype
tumorigenesis
• calcification is not obvious in these tumors
• molar enamel has a hyperplastic intermedium cell layer exhibiting transition to tumor cells
• tumor is composed of benign, eosinophilic, polygonal-shaped cells with intercellular bridges

craniofacial
• molar enamel has a hyperplastic intermedium cell layer exhibiting transition to tumor cells

growth/size/body
• molar enamel has a hyperplastic intermedium cell layer exhibiting transition to tumor cells

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
phenotype observed in males
N normal phenotype
craniofacial
• molar enamel surface has a rough appearance
• hypoplastic enamel lacks normal prism structure
• enamel thickness is 10-20 um in contrast to 100 um observed in wild-type mice

growth/size/body
• molar enamel surface has a rough appearance
• hypoplastic enamel lacks normal prism structure
• enamel thickness is 10-20 um in contrast to 100 um observed in wild-type mice