dentinogenesis imperfecta type 2
Findings
No curated finding names dentinogenesis imperfecta type 2 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Dentinogenesis imperfecta type 2 (DGI-2) is a rare, severe form of dentinogenesis imperfecta (DGI) and is characterized by weakness and discoloration of all teeth.
Definition from the Mondo Disease Ontology (MONDO:0007441), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
HPO, annotations 2026-09-02
Features
2 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.
- Dentinogenesis imperfectaHPOHP:0000703
- Yellow-brown discoloration of the teethHPOHP:0006286
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
- Definitive · G2P · Autosomal dominant · 2015
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
- Narrower terms (1)
Other names
6 names
Resolves to: dentinogenesis imperfecta type 2
- Also called
- Capdepont teethdentinogenesis imperfecta type 1dentinogenesis imperfecta, Shields type 2dentinogenesis imperfecta, Shields type IIDGI-2DI-2