dentinogenesis imperfecta type 3
Findings
No curated finding names dentinogenesis imperfecta type 3 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Dentinogenesis imperfecta type 3 (DGI-3) is a rare, severe form of dentinogenesis imperfecta (DGI) characterized by opalescent primary and permanent teeth, marked attrition, large pulp chambers, multiple pulp exposure and shell teeth radiographically (i.e. teeth which appear hollow due to dentin hypotrophy).
Definition from the Mondo Disease Ontology (MONDO:0007442), read 2026-09-29. CC BY 4.0.
Features
4 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Anterior open-bite malocclusionHPOHP:0009102
- Dental enamel pitsHPOHP:0009722
- Dentinogenesis imperfectaHPOHP:0000703
- Periapical bone lossHPOHP:0000700
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- HGNC:3054HGNC:3054
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2022
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
6 names
Resolves to: dentinogenesis imperfecta type 3
- Also called
- brandywine type dentinogenesis imperfectadentinogenesis imperfecta Shields type 3Dentinogenesis Imperfecta Type IIIdentinogenesis imperfecta, Shields type 3dentinogenesis imperfecta, Shields type IIIDGI-III