Mutant Dentin Sialophosphoprotein Causes Dentinogenesis Imperfecta.

ABSTRACT: Dentin sialophosphoprotein (DSPP) is an extracellular matrix protein highly expressed by odontoblasts in teeth. DSPP mutations in humans may cause dentinogenesis imperfecta (DGI), an autosomal dominant dentin disorder. We recently generated a mouse model (named "Dspp^P19L/+ mice") that expressed a mutant DSPP in which the proline residue at position 19 was replaced by a leucine residue. We found that the Dspp^P19L/+ and Dspp^P19L/P19L mice at a younger age displayed a tooth phenotype resembling human DGI type III characterized by enlarged dental pulp chambers, while the teeth of older Dspp^P19L/+ and Dspp^P19L/P19L mice had smaller dental pulp chambers mimicking DGI type II. The teeth of Dspp^P19L/+

SUBMITTER: Liang T 

PROVIDER: S-EPMC6616118 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature