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What is Dentinogenesis Imperfecta?

Are you wondering what Dentinogenesis Imperfecta is? Dentinogenesis imperfecta (DGI) is a genetic dentine disorder that affects primary and permanent teeth, making them weaker and causing discoloration.

Dentinogenesis imperfecta is considered as a type of dentin dysplasia and a disorder of tooth development that makes teeth prone to breakage, wear, and loss. Other names used to refer to this condition are DGI and hereditary opalescent dentin. This condition affects an estimated 1 in 6,000 to 8,000 people.

The Dentinogenesis imperfecta is inherited in an autosomal dominant pattern and just one copy of the altered gene in each cell is capable of causing this condition.


There are three different types of Dentinogenesis imperfecta according to their cause:

  • Type 1: this type of dentinogenesis imperfecta affects people who have brittle bones that can be broken very easily due to a genetic condition called osteogenesis imperfecta.
  • Type 2: this type of dentinogenesis imperfecta can affect people that don’t suffer from any other inherited disorders, like osteogenesis imperfecta. Some people with Dentinogenesis imperfecta type 2 have also experienced hearing loss besides dental abnormalities.
  • Type 3: In this type of dentinogenesis imperfecta the teeth acquire a shell-like appearance and multiple pulp exposures. It affects especially the permanent teeth but it’s a rare condition.

While the Dentinogenesis imperfecta type 1 occurs due to a genetic condition called osteogenesis imperfecta, the type 2 and 3 of this condition can affect people that don’t have any inherited disorder. What the dentinogenesis imperfecta type 2 and 3 have in common is that people with any of these conditions present mutations n the DSPP gene.

How is dentinogenesis imperfecta produced?

In the case of the Dentinogenesis imperfecta type 2 and 3, they are produced due to mutations in the DSPP gene. The mutations in this gene affect the normal development of teeth because it is the one that contains the instructions for the production of two of the proteins that are essential for its proper formation. Without the presence of these proteins, the dentin, which is a layer that protects the teeth, doesn’t develop properly. The abnormal development of the dentin produces a soft dentin that leads to weak and discolored teeth that are prone to rapid wear, loss, and decay.

When it comes to Dentinogenesis imperfecta type 1, it is produced by mutations but in different genes (like the COL1A1 or COL1A2 genes). The mutations in these genes also lead to a genetic disorder called Osteogenesis Imperfecta, which is an inherited condition that causes brittle bones.


To treat this condition, restorative and aesthetic treatments can be helpful but this disorder must be treated with a lot of care because due to the brittle of the teeth some common cosmetic procedures can cause even more damage. To treat this genetic disorder crowns can be used to improve the appearance of the teeth. Also, another treatment option can be bonding to cover the weakened teeth and make it look whiter.

Any of these procedures will help to cover the damaged teeth and improve its appearance but the teeth on the inside will still be the same.



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