About   Help   FAQ
Due to maintenance, access to MGI may be intermittent 8:00 AM ET Wed, September 18.
Phenotypes Associated with This Genotype
Genotype
MGI:2673970
Allelic
Composition
Dspptm1Kul/Dspptm1Kul
Genetic
Background
involves: 129/Sv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dspptm1Kul mutation (1 available); any Dspp mutation (37 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Tooth abnormalities in Dspptm1Kul/Dspptm1Kul mice

cellular
• 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
• 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
• 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
• 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
• 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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
09/10/2019
MGI 6.14
The Jackson Laboratory