Dentin sialoprotein and dentin phosphoprotein have distinct roles in dentin mineralization.

[dentinogenesis imperfecta]

Dentin sialophosphoprotein (DSPP ), a major non-collagenous matrix protein of odontoblasts , is proteolytically cleaved into dentin sialoprotein (DSP ) and dentin phosphoprotein (DPP ). Our previous studies revealed that DSPP null mice display a phenotype similar to human autosomal dominant dentinogenesis imperfecta , in which teeth have widened predentin and irregular dentin mineralization resulting in sporadic unmineralized areas in dentin and frequent pulp exposure . Earlier in vitro studies suggested that DPP , but not DSP , plays a significant role in initiation and maturation of dentin mineralization . However , the precise in vivo roles of DSP and DPP are far from clear . Here we report the generation of DPPcKO mice , in which only DSP is expressed in a DSPP null background , resulting in a conditional DPP knockout . DPPcKO teeth show a partial rescue of the DSPP null phenotype with the restored predentin width , an absence of irregular unmineralized areas in dentin , and less frequent pulp exposure . Micro-computed tomography (micro-CT ) analysis of DPPcKO molars further confirmed this partial rescue with a significant recovery in the dentin volume , but not in the dentin mineral density . These results indicate distinct roles of DSP and DPP in dentin mineralization , with DSP regulating initiation of dentin mineralization , and DPP being involved in the maturation of mineralized dentin .

Diseases
Validation

Diseases presenting "restored predentin width, an absence" symptom

dentinogenesis imperfecta

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