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Phenotypes Associated with This Genotype
Genotype
MGI:5546382
Allelic
Composition
Tg(KRT14-Hmgn2)#Baam/0
Genetic
Background
Not Specified
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phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
• 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

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 ID Ref(s)
Axenfeld-Rieger Syndrome, Type 1; RIEG1 180500 J:203117


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last database update
04/26/2016
MGI 6.03
The Jackson Laboratory