mortality/aging
• underrepresented at weaning
|
craniofacial
• the lower first molars show significant attrition and formation of reparative dentin
|
• at E13.5 the size of the mesenchymal cell condensations underlying the posterior part of the dental lamina is reduced, but the dental papilla of the first molar has formed
|
• irregular dentin formation is indicated by the presence of 'contour lines of Owen'
|
• in the upper incisors ameloblasts are present at 2 months of age but absent by 5 months
• in the upper incisors ameloblasts fail to elongate and produce only a thin layer of pre-enamel
|
• lower third molars and incisors fail to erupt and other teeth in the lower jaw may also fail to erupt
|
• at P0 all third molars are arrested prior to the bud stage and the lower second molars are arrested at the bud stage
• at P0 the lower first molars have reached the bell stage but lack polarization and alignment of the ameloblasts and odontoblasts
|
• at P0 the upper first and second molars are mostly growth retarded
|
• the upper incisors lack enamel; however enamel formation in the molars is normal
|
• the lower first molars show significant attrition
|
• more severe tooth loss than in Pax9tm1Hpt homozygotes with all mice lacking at least 4 teeth (oligodontia) and some lacking all lower teeth
|
• all mice lack lower incisors
|
• the upper third and lower second and third molars are unilaterally or bilaterally absent
|
growth/size/body
• the lower first molars show significant attrition and formation of reparative dentin
|
• at E13.5 the size of the mesenchymal cell condensations underlying the posterior part of the dental lamina is reduced, but the dental papilla of the first molar has formed
|
• irregular dentin formation is indicated by the presence of 'contour lines of Owen'
|
• in the upper incisors ameloblasts are present at 2 months of age but absent by 5 months
• in the upper incisors ameloblasts fail to elongate and produce only a thin layer of pre-enamel
|
• lower third molars and incisors fail to erupt and other teeth in the lower jaw may also fail to erupt
|
• at P0 all third molars are arrested prior to the bud stage and the lower second molars are arrested at the bud stage
• at P0 the lower first molars have reached the bell stage but lack polarization and alignment of the ameloblasts and odontoblasts
|
• at P0 the upper first and second molars are mostly growth retarded
|
• the upper incisors lack enamel; however enamel formation in the molars is normal
|
• the lower first molars show significant attrition
|
• more severe tooth loss than in Pax9tm1Hpt homozygotes with all mice lacking at least 4 teeth (oligodontia) and some lacking all lower teeth
|
• all mice lack lower incisors
|
• the upper third and lower second and third molars are unilaterally or bilaterally absent
|
skeleton
• the lower first molars show significant attrition and formation of reparative dentin
|
• at E13.5 the size of the mesenchymal cell condensations underlying the posterior part of the dental lamina is reduced, but the dental papilla of the first molar has formed
|
• irregular dentin formation is indicated by the presence of 'contour lines of Owen'
|
• in the upper incisors ameloblasts are present at 2 months of age but absent by 5 months
• in the upper incisors ameloblasts fail to elongate and produce only a thin layer of pre-enamel
|
• lower third molars and incisors fail to erupt and other teeth in the lower jaw may also fail to erupt
|
• at P0 all third molars are arrested prior to the bud stage and the lower second molars are arrested at the bud stage
• at P0 the lower first molars have reached the bell stage but lack polarization and alignment of the ameloblasts and odontoblasts
|
• at P0 the upper first and second molars are mostly growth retarded
|
• the upper incisors lack enamel; however enamel formation in the molars is normal
|
• the lower first molars show significant attrition
|
• more severe tooth loss than in Pax9tm1Hpt homozygotes with all mice lacking at least 4 teeth (oligodontia) and some lacking all lower teeth
|
• all mice lack lower incisors
|
• the upper third and lower second and third molars are unilaterally or bilaterally absent
|
endocrine/exocrine glands
N |
• thymus morphology is normal and no signs of defective parathyroid or ultimobranchial body function (as shown by normal calcium and phosphate homeostasis) are seen
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
tooth agenesis | DOID:0050591 |
OMIM:106600 OMIM:150400 OMIM:313500 OMIM:602639 OMIM:604625 OMIM:610926 OMIM:PS106600 |
J:104121 |