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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TENM1
teneurin transmembrane protein 1
Chromosome X · Xq25
NCBI Gene: 10178Ensembl: ENSG00000009694.14HGNC: HGNC:8117UniProt: B7ZMH4
42PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedTranscription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
signaling receptor bindingnucleuscytoplasmendoplasmic reticulumlateral epicondylitisMoyamoya diseasesensorineural hearing lossSensorineural hearing impairment
✦AI Summary

TENM1 (teneurin transmembrane protein 1) is a membrane protein that plays critical roles in synaptic development and transcriptional regulation. In the olfactory system, TENM1 functions in synaptic-partner-matching between olfactory sensory neuron axons and target projection neurons, and is involved in synapse organization 1. The protein is essential for proper olfactory function, as TENM1 mutations are associated with congenital general anosmia, a complete inability to sense odors 1. TENM1 operates within a transcriptional regulatory network, being directly regulated by EMX2 and functioning during embryonic pattern formation and morphogenesis 2. In cancer contexts, TENM1 demonstrates complex roles with potential tumor-promoting functions in papillary thyroid carcinoma (PTC), where overexpression is associated with disease progression, extrathyroidal invasion, BRAF V600E mutations, and advanced staging 3. TENM1 expression is regulated by miR-486, which targets TENM1 to inhibit cell proliferation, invasion, and migration through modulation of ERK and Akt signaling pathways and epithelial-mesenchymal transition 4. The protein has emerged as a potential prognostic biomarker in PTC, with high expression correlating with poor outcomes 5.

Sources cited
1
TENM1 functions in synaptic-partner-matching in olfactory system and mutations cause congenital general anosmia
PMID: 27040985
2
TENM1 is directly regulated by EMX2 and expressed during embryonic pattern formation and morphogenesis
PMID: 26896429
3
TENM1 overexpression is associated with papillary thyroid carcinoma progression and adverse clinical features
PMID: 28004221
4
miR-486 targets TENM1 to regulate cell proliferation, invasion, and migration via ERK/Akt pathways
PMID: 31646573
5
TENM1 serves as a prognostic biomarker in papillary thyroid carcinoma
PMID: 36193505
Disease Associationsⓘ20
lateral epicondylitisOpen Targets
0.26Weak
Moyamoya diseaseOpen Targets
0.26Weak
Sensorineural hearing impairmentOpen Targets
0.12Weak
sensorineural hearing lossOpen Targets
0.12Weak
glioblastoma multiformeOpen Targets
0.10Suggestive
Hemoglobin E - beta-thalassemiaOpen Targets
0.07Suggestive
hemoglobin E-beta-thalassemia syndromeOpen Targets
0.07Suggestive
hemoglobin D diseaseOpen Targets
0.06Suggestive
hereditary persistence of fetal hemoglobin-sickle cell disease syndromeOpen Targets
0.06Suggestive
dominant beta-thalassemiaOpen Targets
0.05Suggestive
Hereditary persistence of fetal hemoglobin - beta-thalassemiaOpen Targets
0.05Suggestive
delta-beta-thalassemiaOpen Targets
0.05Suggestive
Hemoglobin C - beta-thalassemiaOpen Targets
0.05Suggestive
hemoglobin C-beta-thalassemia syndromeOpen Targets
0.05Suggestive
Alpha-thalassemia - myelodysplastic syndromeOpen Targets
0.05Suggestive
alpha-thalassemia-myelodysplastic syndromeOpen Targets
0.05Suggestive
hemoglobin E diseaseOpen Targets
0.05Suggestive
Blackfan-Diamond anemiaOpen Targets
0.05Suggestive
hemoglobin H diseaseOpen Targets
0.05Suggestive
dehydrated hereditary stomatocytosisOpen Targets
0.05Suggestive
Pathogenic Variants1
NM_001163278.2(TENM1):c.4874T>G (p.Leu1625Arg)Likely pathogenic
Moyamoya angiopathy
☆☆☆☆→ Residue 1625
View on ClinVar ↗
Related Genes
ADGRL3Protein interaction99%ADGRL1Protein interaction99%ADGRL2Protein interaction99%SORBS1Protein interaction94%TENM2Shared pathway25%CXCL1Shared pathway18%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
57%
Lung
12%
Brain
10%
Heart
3%
Ovary
1%
Gene Interaction Network
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TENM1ADGRL3ADGRL1ADGRL2SORBS1TENM2CXCL1
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9UKZ4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.27Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.20 [0.15–0.27]
RankingsWhere TENM1 stands among ~20K protein-coding genes
  • #9,949of 20,598
    Most Researched42
  • #5,155of 5,498
    Most Pathogenic Variants1
  • #879of 17,882
    Most Constrained (LOEUF)0.27 · top 5%
Genes detectedTENM1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A role for TENM1 mutations in congenital general anosmia.
PMID: 27040985
Clin Genet · 2016
1.00
2
HOXA10, EMX2 and TENM1 expression in the mid-secretory endometrium of infertile women with a Müllerian duct anomaly.
PMID: 26896429
Reprod Biomed Online · 2016
0.90
3
Screening and validation of lymph node metastasis risk-factor genes in papillary thyroid carcinoma.
PMID: 36465624
Front Endocrinol (Lausanne) · 2022
0.80
4
Overexpression of teneurin transmembrane protein 1 is a potential marker of disease progression in papillary thyroid carcinoma.
PMID: 28004221
Clin Exp Med · 2017
0.70
5
Teneurins: Role in Cancer and Potential Role as Diagnostic Biomarkers and Targets for Therapy.
PMID: 33652578
Int J Mol Sci · 2021
0.60