dentin dysplasia type II
Findings
No curated finding names dentin dysplasia type II yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Dentin dysplasia type II (DD-II) is a rare mild form of dentin dysplasia (DD) characterized by normal tooth roots but abnormal primary dentition.
Definition from the Mondo Disease Ontology (MONDO:0007437), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Infantile onset
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 imperfecta limited to primary teethHPOHP:0011060
- 7 of 7 reported patients
- Thistle tube shaped pulpHPOHP:0033790
- 1 of 1 reported patient
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
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
- A kind of
Other names
2 names
Resolves to: dentin dysplasia type II
- Also called
- DD-IIDTDP2