DSPP(redirected from DFNA39)
DSPPA gene on chromosome 4q21.3 that encodes one of the two main proteins of the dentin extracellular matrix, which is secreted as a preproprotein by odontoblasts and cleaved into dentin sialoprotein and dentin phosphoprotein. Dentin phosphoprotein is thought to be
involved in the biomineralisation process of dentin.
DSPP mutations are associated with dentinogenesis imperfecta-1, which in some patients is combined with autosomal dominant form of deafness.