TENM3
Chr 4ARteneurin transmembrane protein 3
Also known as: MCOPCB9, MCOPS15, ODZ3, TEN3, TNM3, Ten-m3, ten-3
This gene encodes a teneurin transmembrane protein that regulates neural connectivity establishment, controls precise topographic projections in the hippocampus, and is required for proper dendrite morphogenesis and axon targeting in the visual system. Autosomal recessive mutations cause syndromic microphthalmia with or without coloboma, sometimes associated with developmental dysplasia of the hip. The gene is highly constrained against loss-of-function variants (pLI >0.99), indicating that heterozygous loss-of-function is likely not tolerated.
Primary Disease Associations & Inheritance
Some data sources returned errors (1)
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Population Genetics & Constraint
gnomAD v4 — loss-of-function & missense intolerance
Highly LoF-intolerant (top ~10% of genes)
Highly missense-constrained (top ~0.1%)
Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.
The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.
Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.
ClinVar Variant Classifications
0 submitted variants in ClinVar
Protein Context — Lollipop Plot
TENM3 · protein map & ClinVar variants
Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.
3D Protein StructureAlphaFold
External Resources
Links to major genomics databases and tools
Clinical Trials
Active and recruiting trials from ClinicalTrials.gov
No active trials found for this gene.
Search ClinicalTrials.gov →External Resources
Links to major genomics databases and tools