Dentin sialophosphoprotein (DSPP)

Dentin sialophosphoprotein is a large, non-collagenous extracellular matrix precursor protein most abundantly expressed by odontoblasts during tooth formation and is critical for proper mineralization and structure of mineralized dentin[1][2][3][4][5]. Following secretion, DSPP undergoes proteolytic cleavage, producing three main proteins: dentin sialoprotein (DSP), dentin glycoprotein (DGP—recognized in pigs, not verified in humans), and dentin phosphoprotein (DPP; sometimes called phosphophoryn)[1][2][3][4][5]. These cleavage products regulate dentin mineralization by organizing and initiating hydroxyapatite deposition within the collagen matrix, with DSP and DPP serving different structural and regulatory roles[3][4]. Inherited mutations in the DSPP gene are the primary cause of dentinogenesis imperfecta types II and III and dentin dysplasia type II; some DSPP mutations are also associated with autosomal dominant non-syndromic hearing loss (DFNA39)[1][3][5]. DSPP is not a standard therapeutic target (such as a receptor, enzyme, or transporter) and is not modulated by drugs; rather, it is notable in medical genetics as the causal gene for several inherited dental and, occasionally, auditory phenotypes[1][3][5].

Other names

Dentin phosphoproteinDentin sialoproteinDPPDSPDGP (dentin glycoprotein)DMP3 (deprecated, sometimes used)Dentin phosphophorynDentin phosphorynDeafness, autosomal dominant 39 (DFNA39; relates to linked phenotype)DGI1 (linked to disease phenotype)