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GeneE
25 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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TECR
trans-2,3-enoyl-CoA reductase
Chromosome 19 · 19p13.12
NCBI Gene: 9524Ensembl: ENSG00000099797.16HGNC: HGNC:4551UniProt: B3KSQ1
175PubMed Papers
21Diseases
0Drugs
0Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingsphingolipid metabolic processfatty acid elongationvery long-chain fatty acid biosynthetic processneurodegenerative diseaseautosomal recessive non-syndromic intellectual disabilityjaw diseasesystemic lupus erythematosus
✦AI Summary

TECR (trans-2,3-enoyl-CoA reductase) is an endoplasmic reticulum-bound enzyme catalyzing the final reduction step in long-chain fatty acid elongation 1. It reduces trans-2,3-enoyl-CoA intermediates to acyl-CoA substrates, enabling addition of 2-carbon units per cycle to produce very-long-chain fatty acids (VLCFAs) with diverse biological functions 1. TECR also participates in sphingosine 1-phosphate metabolism, converting trans-2-hexadecenoyl-CoA to palmitoyl-CoA 2. The enzyme functions within a membrane-bound complex involving HACD1/2 and fatty acid elongases, with critical catalytic residues including Y248 that provides protons to substrates 3. Beyond canonical lipid synthesis, TECR promotes novel, TECR-dependent nonapoptotic cancer cell death distinct from ferroptosis, necroptosis, and pyroptosis, with tegavivint (a clinical drug candidate) activating this mechanism via palmitate synthesis 4. Pathologically, homozygous TECR variants cause intellectual developmental disorder with expanded phenotypes including dolicocephaly and corpus callosum dysgenesis, particularly identified in Hutterite populations 5. TECR expression is also dysregulated in Kaposi's sarcoma and esophageal cancer stem cells 67, and epigenetic regulation of TECR correlates with circulating adiponectin levels in lipid metabolism 8.

Sources cited
1
TECR catalyzes the last reaction of fatty acid elongation cycle, reducing trans-2,3-enoyl-CoA to allow 2-carbon addition and VLCFA production
PMID: 12482854
2
TECR participates in sphingosine 1-phosphate metabolism, converting trans-2-hexadecenoyl-CoA to palmitoyl-CoA
PMID: 25049234
3
TECR catalytic residue Y248 provides protons to substrates and coordinates with fatty acid elongases in the elongation cycle complex
PMID: 38224948
4
TECR activation by tegavivint triggers nonapoptotic cancer cell death distinct from ferroptosis/necroptosis/pyroptosis via palmitate synthesis
PMID: 40419770
5
Homozygous TECR variants cause intellectual developmental disorder with dolicocephaly and corpus callosum dysgenesis, particularly in Hutterite populations
PMID: 35709344
6
TECR is downregulated in Kaposi's sarcoma tissues and is among dysregulated RNA-binding proteins in KS
PMID: 40016701
7
TECR is among differentially methylated genes in esophageal cancer stem cells
PMID: 33357046
8
DNA methylation at cg02561343 associates with TECR expression in subcutaneous adipose tissue and correlates with circulating adiponectin levels
PMID: 41793944
Disease Associationsⓘ21
neurodegenerative diseaseOpen Targets
0.50Moderate
autosomal recessive non-syndromic intellectual disabilityOpen Targets
0.41Moderate
jaw diseaseOpen Targets
0.25Weak
systemic lupus erythematosusOpen Targets
0.21Weak
smoking initiationOpen Targets
0.14Weak
risk-taking behaviourOpen Targets
0.14Weak
esophageal squamous cell carcinomaOpen Targets
0.07Suggestive
annular pancreasOpen Targets
0.06Suggestive
hypertriglyceridemia 2Open Targets
0.06Suggestive
familial hypercholesterolemiaOpen Targets
0.05Suggestive
Hypoplastic pancreas-intestinal atresia-hypoplastic gallbladder syndromeOpen Targets
0.05Suggestive
Combined hyperlipidemiaOpen Targets
0.04Suggestive
thyroid hormone metabolism, abnormal, 2Open Targets
0.04Suggestive
pachyonychia congenitaOpen Targets
0.04Suggestive
sitosterolemia 2Open Targets
0.04Suggestive
homozygous familial hypercholesterolemiaOpen Targets
0.04Suggestive
gastric adenocarcinoma and proximal polyposis of the stomachOpen Targets
0.04Suggestive
gastric cancerOpen Targets
0.04Suggestive
gastric carcinomaOpen Targets
0.04Suggestive
chronic intestinal pseudoobstructionOpen Targets
0.04Suggestive
Intellectual developmental disorder, autosomal recessive 14UniProt
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HSD17B12Protein interaction100%SCDProtein interaction93%FADS1Protein interaction92%ERMAPProtein interaction92%ACOT1Protein interaction91%ACOT7Protein interaction90%
Tissue Expression6 tissues
Brain
100%
Liver
62%
Ovary
41%
Bone Marrow
40%
Heart
37%
Lung
37%
Gene Interaction Network
Click a node to explore
TECRHSD17B12SCDFADS1ERMAPACOT1ACOT7
PROTEIN STRUCTURE
Preparing viewer…
PDB2DZJ · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.54Moderately Constrained
pLIⓘ
0.85Intermediate
Observed/Expected LoF0.37 [0.26–0.54]
RankingsWhere TECR stands among ~20K protein-coding genes
  • #2,511of 20,598
    Most Researched175 · top quartile
  • #3,421of 17,882
    Most Constrained (LOEUF)0.54 · top quartile
Genes detectedTECR
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
Tegavivint triggers TECR-dependent nonapoptotic cancer cell death.
PMID: 40419770
Nat Chem Biol · 2025
1.00
2
A clinical drug candidate that triggers non-apoptotic cancer cell death.
PMID: 39989975
Res Sq · 2025
0.90
3
The 3-hydroxyacyl-CoA dehydratase 1/2 form complex with trans-2-enoyl-CoA reductase involved in substrates transfer in very long chain fatty acid elongation.
PMID: 38422897
Biochem Biophys Res Commun · 2024
0.80
4
Exploration of RNA-binding proteins identified RPS27 as a potential regulator associated with Kaposi's sarcoma development.
PMID: 40016701
BMC Cancer · 2025
0.70
5
Key Considerations in Ensuring a Safe Regional Telehealth Care Model: A Systematic Review.
PMID: 33956524
Telemed J E Health · 2022
0.64