Article
Dentin defects caused by a Dspp-1 frameshift mutation are associated with the activation of autophagy.
Scientific reports - 19 Apr 2023
Liang Tian, Smith Charles E, Hu Yuanyuan, Zhang Hong, Zhang Chuhua, Xu Qian, Lu Yongbo, Qi Ling, Hu Jan C-C, Simmer James P
Abstract excerpt
Dentin sialophosphoprotein (DSPP) is primarily expressed by differentiated odontoblasts (dentin-forming cells), and transiently expressed by presecretory ameloblasts (enamel-forming cells). Disease-causing DSPP mutations predominantly fall into two categories: 5' mutations affecting targeting and trafficking, and 3' - 1 frameshift mutations converting the repetitive, hydrophilic, acidic C-terminal domain into a...
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