dentin dysplasia type I
MONDO:0007436Mondo
Findings
No curated finding names dentin dysplasia type I yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Dentin dysplasia type I (DD-I) is a rare form of dentin dysplasia (DD) characterized by sharp conical short roots or rootless teeth.
Definition from the Mondo Disease Ontology (MONDO:0007436), read 2026-09-29. CC BY 4.0.
Genes
3 genes
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.
- SMOC2HGNC:20323
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal recessive · 2020
- Strong · G2P · Autosomal recessive · 2015
- Limited · Ambry Genetics · Autosomal recessive · 2022
- HGNC:24809HGNC:24809
- Supportive · Orphanet · Autosomal dominant · 2021
- VPS4BHGNC:10895
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
- A kind of
Other names
4 names
Resolves to: dentin dysplasia type I
- Also called
- DD-Identin dysplasia, type i, with microdontia and misshapen teethDTDP1radicular dentin dysplasia