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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ECHDC3
enoyl-CoA hydratase domain containing 3
Chromosome 10 · 10p14
NCBI Gene: 79746Ensembl: ENSG00000134463.16HGNC: HGNC:23489UniProt: A0A140VKF9
15PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
positive regulation of cellular response to insulin stimulusmitochondrionenoyl-CoA hydratase activityhydro-lyase activityAlzheimer diseaselate-onset Alzheimers diseasealcohol drinkingdementia
✦AI Summary

ECHDC3 is a mitochondrial enoyl-CoA hydratase involved in fatty acid metabolism and insulin sensitivity regulation. The gene contains conserved hydro-lyase domains and localizes to mitochondria where it participates in fatty acid metabolic processes 1. Mechanistically, ECHDC3 expression in adipose tissue is genetically regulated by cis-eQTL variants and directly impacts insulin sensitivity. ECHDC3 silencing in adipocytes significantly reduces insulin-stimulated glucose uptake and Akt phosphorylation, with downstream effects on γ-linolenate biosynthesis pathways and insulin resistance-associated genes 1. ECHDC3 expression correlates positively with the Matsuda insulin sensitivity index across African American and European populations 1. Clinical relevance spans multiple diseases. ECHDC3 variants associate with white matter hyperintensity and regulate right hippocampal microstructural integrity in type 2 diabetes patients, mediating effects on episodic memory decline 2. The rs7920721 variant represents a genome-wide significant Alzheimer's disease susceptibility locus, with altered ECHDC3 expression observed in AD brains compared to controls 3. In Alzheimer's disease progression, ECHDC3 expression negatively associates with structural brain atrophy in the thalamus, cerebellum, and frontal regions 4. Additionally, ECHDC3 upregulation correlates with coronary artery lesion progression and serves as a prognostic biomarker in kidney renal clear cell carcinoma 56.

Sources cited
1
ECHDC3 expression in adipose tissue is genetically regulated and directly controls insulin sensitivity through effects on glucose uptake and Akt phosphorylation
PMID: 31010960
2
ECHDC3 rs11257311 variant regulates hippocampal white matter microstructure and mediates type 2 diabetes effects on short-term memory
PMID: 38070593
3
ECHDC3 rs7920721 is a genome-wide significant Alzheimer's disease risk variant with altered expression in AD brains
PMID: 25862742
4
ECHDC3 expression negatively associates with structural brain atrophy across the clinical AD spectrum
PMID: 39554844
5
ECHDC3 mRNA upregulation correlates with coronary artery lesion progression and monounsaturated fatty acid levels
PMID: 27586541
6
ECHDC3 is part of a cigarette smoke-related gene signature prognostic for kidney renal clear cell carcinoma
PMID: 35140327
7
ECHDC3 is identified as a key driver gene in acute peanut allergic reactions through network analysis
PMID: 29203772
Disease Associationsⓘ20
Alzheimer diseaseOpen Targets
0.44Moderate
late-onset Alzheimers diseaseOpen Targets
0.37Weak
alcohol drinkingOpen Targets
0.31Weak
dementiaOpen Targets
0.28Weak
acute myeloid leukemiaOpen Targets
0.06Suggestive
Abnormality of limbsOpen Targets
0.05Suggestive
cervical carcinomaOpen Targets
0.05Suggestive
response to antihypertensive drugOpen Targets
0.04Suggestive
SepsisOpen Targets
0.02Suggestive
injuryOpen Targets
0.02Suggestive
placenta praeviaOpen Targets
0.02Suggestive
familial hemolytic anemiaOpen Targets
0.02Suggestive
Parkinson diseaseOpen Targets
0.02Suggestive
transitional cell carcinoma of kidneyOpen Targets
0.02Suggestive
cardiovascular diseaseOpen Targets
0.01Suggestive
leukemiaOpen Targets
0.01Suggestive
bacteriemiaOpen Targets
0.01Suggestive
adrenal cortex carcinomaOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
breast ductal carcinoma in situOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CYP4B1Shared pathway100%PCCAShared pathway100%ACBD7Shared pathway100%AASDHShared pathway100%ACBD4Shared pathway100%PEDS1Shared pathway50%
Tissue Expression6 tissues
Liver
100%
Heart
23%
Lung
17%
Ovary
7%
Brain
4%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
ECHDC3CYP4B1PCCAACBD7AASDHACBD4PEDS1
PROTEIN STRUCTURE
Preparing viewer…
PDB2VX2 · 2.30 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.61LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.06 [0.71–1.61]
RankingsWhere ECHDC3 stands among ~20K protein-coding genes
  • #15,542of 20,598
    Most Researched15
  • #15,734of 17,882
    Most Constrained (LOEUF)1.61
Genes detectedECHDC3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Consistent genes associated with structural changes in clinical Alzheimer's disease spectrum.
PMID: 39554844
Front Neurosci · 2024
1.00
2
ECHDC3 Variant Regulates the Right Hippocampal Microstructural Integrity and Verbal Memory in Type 2 Diabetes Mellitus.
PMID: 38070593
Neuroscience · 2024
0.90
3
The relationship of the oleic acid level and ECHDC3 mRNA expression with the extent of coronary lesion.
PMID: 27586541
Lipids Health Dis · 2016
0.80
4
Polygenic Overlap Between C-Reactive Protein, Plasma Lipids, and Alzheimer Disease.
PMID: 25862742
Circulation · 2015
0.70
5
A genome wide meta-analysis study for identification of common variation associated with breast cancer prognosis.
PMID: 25526632
PLoS One · 2014
0.60