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tooth agenesis, selective, 9 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

tooth agenesis, selective, 9

MONDO:0014999

Also known as:GREM2 tooth agenesisSTHAG9tooth agenesis caused by mutation in GREM2

Overview

Any tooth agenesis in which the cause of the disease is a mutation in the GREM2 gene.

GeneticsAutosomal dominant · GREM2 (chr 1)

Clinical Presentation

Features include very common findings: Selective tooth agenesis; and sometimes findings: Microdontia and Taurodontia.

Explore clinical features

Genetic Landscape

Assembled from HGNC gene records, ClinGen gene-disease validity evidence, and ClinVar pathogenic variant data. Validity classifications reflect the strength of published evidence linking each gene to this disease.

GREM2 encodes gremlin 2, DAN family BMP antagonist (168 aa). Cytokine that inhibits the activity of BMP2 and BMP4 in a dose-dependent manner, and thereby modulates signaling by BMP family members. Highest expression in Colon Sigmoid (29.5 TPM) and Colon Transverse (19.8 TPM).

Tooth agenesis, selective, 9 has limited evidence linking it to mutations in the GREM2 gene on chromosome 1.

GREM2 is classified as a druggable target (Druggable Genome category) with score 3.5.

Diagnosis

Genetic testing for GREM2 is available. Testing is considered research-grade for diagnosis.

Prognosis

Phenotype severity distribution: 1 very common feature.

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, 9.

15 publications have been identified in PubMed for tooth agenesis, selective, 9. Research spans Case Report / Case Series (33%), Epidemiology / Natural History (27%), and Review / Meta-Analysis (13%).

Research Type	Count	% of Total
Patient case studies	5	33%

Data assembled from 6 of 12 sources · Last updated Sep 19, 2026, 6:43 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:617275

Questions for Your Care Team

→Should I pursue genetic testing for GREM2, and what would the results mean for my family?
→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, 9

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

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Questions for Your Care Team

Questions for Your Care Team

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