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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ACADL
acyl-CoA dehydrogenase long chain
Chromosome 2 · 2q34
NCBI Gene: 33Ensembl: ENSG00000115361.9HGNC: HGNC:88UniProt: P28330
41PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
long-chain fatty acyl-CoA dehydrogenase activityprotein bindingmitochondrionregulation of cholesterol metabolic processlong chain acyl-CoA dehydrogenase deficiencyvery long chain acyl-CoA dehydrogenase deficiencyhepatocellular carcinomaneoplasm
✦AI Summary

ACADL (acyl-CoA dehydrogenase long chain) is a mitochondrial enzyme that catalyzes the first step of long-chain fatty acid β-oxidation, converting acyl-CoA thioesters to trans-2-enoyl-CoA using electron transfer flavoprotein as an electron acceptor 1. The enzyme preferentially acts on saturated and unsaturated fatty acyl-CoAs with 6-24 carbons, with optimal activity on 8-18 carbon chains, and likely metabolizes bulky substrates including branched-chain fatty acids and sterol derivatives. ACADL is the predominant long-chain acyl-CoA dehydrogenase in lung type 2 alveolar epithelial cells, where it regulates surfactant production and controls alveolar inflammation 2. Functionally, ACADL plays a critical immunometabolic role: deleting Acadl in alveolar cells restricts neutrophilic inflammation during acute lung injury by reducing CXCL2 chemokine production 2. In hepatocellular carcinoma, ACADL functions as a tumor suppressor whose expression is mechanically regulated by extracellular matrix stiffness through the YAP/TEAD4 transcriptional axis 3. ACADL expression is also dysregulated in metabolic diseases: upregulated in non-alcoholic steatohepatitis livers and aberrantly regulated in diabetic cardiomyopathy hearts, contributing to lipid and branched-chain amino acid metabolic dysfunction 45. These findings establish ACADL as a metabolic hub integrating energy production, immune regulation, and tissue-specific physiological functions.

Sources cited
1
ACADL catalyzes the first reaction of mitochondrial β-oxidation of fatty acids; molecular cloning and chromosome assignment
PMID: 1774065
2
ACADL in lung type 2 alveolar epithelial cells regulates mitochondrial long-chain fatty acid β-oxidation and controls alveolar inflammation in acute lung injury
PMID: 39174557
3
ACADL functions as a tumor suppressor in hepatocellular carcinoma with expression regulated by extracellular matrix stiffness through YAP/TEAD4 axis
PMID: 37151879
4
ACADL (Lcad) expression is upregulated in non-alcoholic steatohepatitis and involved in lipid metabolism dysregulation
PMID: 29273476
5
ACADL is aberrantly regulated in diabetic cardiomyopathy contributing to fatty acid metabolic dysfunction
PMID: 39623870
6
ACADL undergoes lactylation modifications that correlate with altered expression during cardiac metabolic reprogramming in neonatal hearts
PMID: 38479452
Disease Associationsⓘ20
long chain acyl-CoA dehydrogenase deficiencyOpen Targets
0.16Weak
very long chain acyl-CoA dehydrogenase deficiencyOpen Targets
0.12Weak
hepatocellular carcinomaOpen Targets
0.11Weak
neoplasmOpen Targets
0.09Suggestive
lung adenocarcinomaOpen Targets
0.09Suggestive
non-small cell lung carcinomaOpen Targets
0.08Suggestive
insomniaOpen Targets
0.08Suggestive
esophageal squamous cell carcinomaOpen Targets
0.08Suggestive
acute kidney injuryOpen Targets
0.07Suggestive
breast cancerOpen Targets
0.05Suggestive
glycogen storage disease VIOpen Targets
0.05Suggestive
neonatal intrahepatic cholestasis due to citrin deficiencyOpen Targets
0.05Suggestive
Hyperinsulinism due to short chain 3-hydroxylacyl-CoA dehydrogenase deficiencyOpen Targets
0.04Suggestive
3-hydroxy-3-methylglutaryl-CoA synthase deficiencyOpen Targets
0.04Suggestive
carnitine palmitoyl transferase 1A deficiencyOpen Targets
0.04Suggestive
lung carcinomaOpen Targets
0.04Suggestive
glycogen storage disease IXcOpen Targets
0.04Suggestive
glycogen storage disease due to GLUT2 deficiencyOpen Targets
0.04Suggestive
cardiac hypertrophyOpen Targets
0.04Suggestive
carnitine palmitoyl transferase II deficiency, severe infantile formOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ACOX1Protein interaction100%ACAA2Protein interaction100%ACOX3Protein interaction99%ACADSProtein interaction98%ACADSBProtein interaction98%CPT2Protein interaction98%
Tissue Expression6 tissues
Liver
100%
Ovary
62%
Lung
22%
Heart
7%
Brain
3%
Bone Marrow
0%
Gene Interaction Network
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ACADLACOX1ACAA2ACOX3ACADSACADSBCPT2
PROTEIN STRUCTURE
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PDB8W0Z · 2.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.26LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.97 [0.76–1.26]
RankingsWhere ACADL stands among ~20K protein-coding genes
  • #9,968of 20,598
    Most Researched41
  • #13,307of 17,882
    Most Constrained (LOEUF)1.26
Genes detectedACADL
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A 20-year Clinical and Genetic Neuromuscular Cohort Analysis in Lebanon: An International Effort.
PMID: 34602496
J Neuromuscul Dis · 2022
1.00
2
Bulk and single-cell transcriptome profiling reveal extracellular matrix mechanical regulation of lipid metabolism reprograming through YAP/TEAD4/ACADL axis in hepatocellular carcinoma.
PMID: 37151879
Int J Biol Sci · 2023
0.90
3
Alveolar epithelial cells mitigate neutrophilic inflammation in lung injury through regulating mitochondrial fatty acid oxidation.
PMID: 39174557
Nat Commun · 2024
0.80
4
A systematic review of p53 regulation of oxidative stress in skeletal muscle.
PMID: 29298131
Redox Rep · 2018
0.70
5
Gasdermin D plays a key role as a pyroptosis executor of non-alcoholic steatohepatitis in humans and mice.
PMID: 29273476
J Hepatol · 2018
0.60