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Genetic and Rare Diseases Information Center (GARD)

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Dentinogenesis imperfecta Shields type 3

Other Names for this Disease
  • Brandywine type dentinogenesis imperfecta
  • Dentinogenesis imperfecta type III
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Your Question

My daughters are affected by dentinogenesis imperfecta type III. I have taken them to many dentists in an attempt to get them help. Can you provide information regarding how to correct their teeth?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is dentinogenesis imperfecta type III?

Dentinogenesis imperfecta type III is one of five distinct, hereditary disorders of dentin (the bone-like substance that makes up most of the tooth) development affecting the teeth.[1] This condition causes the teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. Dentinogenesis imperfecta type III is caused by mutations in the DSPP gene which are inherited in an autosomal dominant fashion.[2] Treatment, which may begin as early as infancy, usually continues into adulthood with a number of options including the use of crowns, over-dentures and dental implants depending on the age of the patient and the condition of the dentition.[3] Individuals with dentinogenesis imperfecta type III appear to be limited, in large measure, to a population in the region around Brandywine in southern Maryland.[1]
Last updated: 2/19/2009

What causes dentinogenesis imperfecta type III?

Mutations in the DSPP gene have been identified in individuals with dentinogenesis imperfecta type III. The DSPP gene provides instructions for making three proteins that are essential for normal tooth development. These proteins are involved in the formation of dentin, which is a bone-like substance that makes up the protective middle layer of each tooth. DSPP mutations alter the proteins made from the gene, leading to the production of abnormally soft dentin. Teeth with defective dentin are discolored, weak, and more likely to decay and break.[2]
Last updated: 2/19/2009

How do people inherit dentinogenesis imperfecta type III?

Dentinogenesis imperfecta type III is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person has one parent with the condition.[2]

Last updated: 2/19/2009

How might dentinogenesis imperfecta type III be treated?

The aims of treatment are to remove sources of infection or pain, restore aesthetics and protect posterior teeth from wear. Treatment varies according to the age of the patient, severity of the problem and the presenting complaint.[4] 

Crowns, caps or other forms of dental care are the most commonly used treatments. Dentures or dental implants may be necessary if the majority of teeth are lost.[5]

More detailed information regarding the treatment of dentinogenesis imperfecta can be found by visiting the following web link:

Last updated: 5/19/2010