Enamel Defects Associated With Dentin Sialophosphoprotein Mutation in Mice.

ABSTRACT: Dentin sialophosphoprotein (DSPP) is an extracellular matrix protein that is highly expressed in odontoblasts, but only transiently expressed in presecretory ameloblasts during tooth development. We previously generated a knockin mouse model expressing a mouse equivalent (DSPP, p.P19L) of human mutant DSPP (p.P17L; referred to as "Dspp^P19L/+ "), and reported that Dspp^P19L/+ and Dspp^P19L/P19L mice manifested a dentin phenotype resembling human dentinogenesis imperfecta (DGI). In this study, we analyzed pathogenic effects of mutant P19L-DSPP on enamel development in Dspp^P19L/+ and Dspp^P19L/P19L mice. Micro-Computed Tomography (μCT) analyses of 7-week-old mouse mandibular incisors showed that Dspp

SUBMITTER: Liang T 

PROVIDER: S-EPMC8497714 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature