Mouse Genome Informatics
tg
    Tg(KRT14-Hmgn2)#Baam/0
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
craniofacial
• shorter upper and lower incisors at P17
• teeth have a transparent texture and chalky white surface, indicating lack of enamel deposition and mineralization
• residual Amel protein tends to aggregate and form a patch-like enamel layer that is severely depleted with respect to both deposition and calcification compared to wild-type
• the irregular pattern of enamel formation resembles the human enamel hypoplasia seen in Axenfeld-Rieger Syndrome patients
• however, defects in the differentiation of ameloblasts in the lower incisors is not seen at P4

growth/size/body
• shorter upper and lower incisors at P17
• teeth have a transparent texture and chalky white surface, indicating lack of enamel deposition and mineralization
• residual Amel protein tends to aggregate and form a patch-like enamel layer that is severely depleted with respect to both deposition and calcification compared to wild-type
• the irregular pattern of enamel formation resembles the human enamel hypoplasia seen in Axenfeld-Rieger Syndrome patients
• however, defects in the differentiation of ameloblasts in the lower incisors is not seen at P4

Mouse Models of Human Disease
OMIM IDRef(s)
Axenfeld-Rieger Syndrome, Type 1; RIEG1 180500 J:203117