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tooth agenesis, selective, 4 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

tooth agenesis, selective, 4

MONDO:0007881

Also known as:WNT10A tooth agenesistooth agenesis caused by mutation in WNT10Atooth agenesis, selective, type 4

Overview

Any tooth agenesis in which the cause of the disease is a mutation in the WNT10A gene.

GeneticsAutosomal recessive, Autosomal dominant · WNT10A (chr 2)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: Agenesis of permanent teeth; and common findings: Sparse body hair, Short eyelashes, Sparse eyebrow, and Dystrophic fingernails and others. 16 total HPO annotations.

Organ System	Phenotype Count	Example Features
Skin	5	Palmar hyperkeratosis, Dry skin, Palmoplantar hyperhidrosis

Genetic Landscape

WNT10A function has not been fully characterized.

Tooth agenesis, selective, 4 is associated with mutations in the WNT10A gene on chromosome 2.

Diagnosis

Genetic testing for WNT10A is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for tooth agenesis, selective, 4 has been reported in the published literature.

Prognosis

Phenotype severity distribution: 1 always present feature, 8 common features.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for tooth agenesis, selective, 4.

24 publications have been identified in PubMed for tooth agenesis, selective, 4. Research spans Review / Meta-Analysis (36%), Epidemiology / Natural History (23%), and Case Report / Case Series (18%).

Research Type	Count	% of Total
Research summaries	8	36%

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 1:53 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:150400

GARD

Genetic and Rare Diseases Info Center

GARD:18245

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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tooth agenesis, selective, 4

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

tooth agenesis, selective, 4

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications