Mouse Dspp frameshift model of human dentinogenesis imperfecta.

ABSTRACT: Non-syndromic inherited defects of tooth dentin are caused by two classes of dominant negative/gain-of-function mutations in dentin sialophosphoprotein (DSPP): 5' mutations affecting an N-terminal targeting sequence and 3' mutations that shift translation into the - 1 reading frame. DSPP defects cause an overlapping spectrum of phenotypes classified as dentin dysplasia type II and dentinogenesis imperfecta types II and III. Using CRISPR/Cas9, we generated a Dspp^-1fs mouse model by introducing a FLAG-tag followed by a single nucleotide deletion that translated 493 extraneous amino acids before termination. Developing incisors and/or molars from this mouse and a Dspp^P19L mouse were characterized by morphological assessment, bSEM, nanohardness testing, histological analy

SUBMITTER: Liang T 

PROVIDER: S-EPMC8526674 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature