Analysis Tools
cellular
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• mutant embryos exhibited a larger proportion of cells undergoing apoptosis in the kidney than controls at E13.5
|
craniofacial
|
• alveolar bone resorption
|
|
• no tooth phenotype until 2-3 months of age
|
|
• pulp cavity enlarged
• pulp cavity exposure begins around 2 months (not in incisors)
• leads to discoloration and degeneration
• leads to roots shortened
• tooth crown worn down to gingiva and alveolar bone resorption
• pulp degeneration in older mice
|
|
• globular dentin with extensive interglobular regions seen in incisors
• collagenous network lining dentinal tubules frequently lacking
• well organized intertubular dentin absent
|
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• irregular mineralization due to abnormal calcospherite coalescence
• high levels of biglycan and decorin interfere with mineralization
|
|
• increased distribution and levels of proteoglycans in predentin
• increased predentin and irregular border with dentin
|
brittle teeth
(
J:84378
)
|
• occasionally the incisors appear broken in older mice
|
renal/urinary system
|
• mutant embryos exhibited a larger proportion of cells undergoing apoptosis in the kidney than controls at E13.5
|
|
• fewer ureteric bud branches at E13.5
|
|
• delay in the maturation of the nephrons is reflected by overall smaller size of the metanephros at E13.5
|
|
• delayed maturation of the nephron elements at E13.5
• less polarized epithelial layers in the nephrons at E13.5
|
|
• malformed and fewer Sshaped bodies in the mutant mouse embryonic kidney at E13.5
|
skeleton
|
• alveolar bone resorption
|
|
• no tooth phenotype until 2-3 months of age
|
|
• pulp cavity enlarged
• pulp cavity exposure begins around 2 months (not in incisors)
• leads to discoloration and degeneration
• leads to roots shortened
• tooth crown worn down to gingiva and alveolar bone resorption
• pulp degeneration in older mice
|
|
• globular dentin with extensive interglobular regions seen in incisors
• collagenous network lining dentinal tubules frequently lacking
• well organized intertubular dentin absent
|
|
• irregular mineralization due to abnormal calcospherite coalescence
• high levels of biglycan and decorin interfere with mineralization
|
|
• increased distribution and levels of proteoglycans in predentin
• increased predentin and irregular border with dentin
|
brittle teeth
(
J:84378
)
|
• occasionally the incisors appear broken in older mice
|
growth/size/body
|
• alveolar bone resorption
|
|
• no tooth phenotype until 2-3 months of age
|
|
• pulp cavity enlarged
• pulp cavity exposure begins around 2 months (not in incisors)
• leads to discoloration and degeneration
• leads to roots shortened
• tooth crown worn down to gingiva and alveolar bone resorption
• pulp degeneration in older mice
|
|
• globular dentin with extensive interglobular regions seen in incisors
• collagenous network lining dentinal tubules frequently lacking
• well organized intertubular dentin absent
|
|
• irregular mineralization due to abnormal calcospherite coalescence
• high levels of biglycan and decorin interfere with mineralization
|
|
• increased distribution and levels of proteoglycans in predentin
• increased predentin and irregular border with dentin
|
brittle teeth
(
J:84378
)
|
• occasionally the incisors appear broken in older mice
|
respiratory system
|
• at E13.5, lungs in the mutant mice have altered morphology of the ductlike structures
• lung duct epithelia are less stratified
• fewer cytoplasmic vesicles in lung ducts, suggesting a reduced secretory activity of the ductular epithelia
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| dentinogenesis imperfecta | DOID:4154 |
OMIM:125490 OMIM:125500 |
J:84378 | |
