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DSPP gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

DSPP gene

The DSPP gene is essential for normal tooth development, and mutations in this gene are linked to hereditary conditions that cause weak, discolored, and brittle teeth.

Normal Function

The DSPP gene provides instructions for making a protein called dentin sialophosphoprotein. This protein is processed into smaller proteins—dentin sialoprotein and dentin phosphoprotein—which are critical components of dentin, the protective middle layer of a tooth. Dentin phosphoprotein is thought to be involved in the mineralization process, where mineral crystals are deposited among collagen fibers to harden the dentin. The DSPP gene is also active in the inner ear, though its role in hearing is not fully understood.

Health Conditions Related to Genetic Changes

Dentinogenesis imperfecta

More than 20 mutations in the DSPP gene have been identified in people with dentinogenesis imperfecta (types II and III). These mutations also cause dentin dysplasia type II. These genetic changes interfere with the production, secretion, or transport of DSPP-derived proteins, resulting in teeth that have abnormally soft dentin. Consequently, these teeth are discolored, weak, and prone to breakage and decay.

Nonsyndromic hearing loss

While the DSPP gene is active in the inner ear, it is unclear if mutations are directly related to the hearing loss observed in some individuals with dentinogenesis imperfecta type II.

Other Names for This Gene

  • dentin glycoprotein
  • dentin phosphophoryn
  • dentin phosphoprotein
  • dentin phosphoryn
  • dentin sialoprotein
  • DFNA39
  • DGI1
  • DGP
  • DPP
  • DSP
  • DSPP_HUMAN
  • DTDP2

Frequently Asked Questions

There is currently no genetic cure for DSPP mutations, so management focuses on protecting the existing teeth and replacing lost ones. Treatment often involves placing full-coverage crowns on the teeth to shield the weak dentin from further wear and fracture. In severe cases where teeth are lost, dental implants or bridges may be necessary. Regular monitoring by a specialist is essential to catch decay or structural failure early.
Yes, prenatal diagnosis is possible if a specific DSPP mutation has already been identified in a family member. This is typically done through chorionic villus sampling (CVS) or amniocentesis to analyze the fetal DNA. If no family history exists, postnatal diagnosis is usually confirmed via genetic sequencing of the DSPP gene, often after a dentist or geneticist observes the characteristic clinical features of the teeth.
Because the dentin is weak, standard brushing may cause excessive wear. Dentists often recommend using a soft-bristled toothbrush and non-abrasive fluoride toothpaste. It is crucial to avoid hard, crunchy, or sticky foods that can crack the teeth. Additionally, wearing a custom-fitted mouthguard during sleep and sports is highly recommended to protect the teeth from grinding and impact, as the structural integrity of the dentin is compromised.
Early signs often appear when the first primary (baby) teeth erupt. Parents may notice that the teeth have a distinct blue-gray, amber, or brownish tint rather than the normal white color. The teeth may also appear unusually translucent, allowing the underlying pulp to show through. As the child begins to chew, the teeth may wear down rapidly or chip easily, which is a key indicator that the dentin layer is abnormally soft and weak.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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