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Aliases for ACADL Gene

Aliases for ACADL Gene

  • Acyl-CoA Dehydrogenase, Long Chain 2 3 5
  • Acyl-Coenzyme A Dehydrogenase, Long Chain 2 3
  • LCAD 3 4
  • EC 63
  • EC 4
  • EC 1.3.99 63
  • ACAD4 3

External Ids for ACADL Gene

Previous GeneCards Identifiers for ACADL Gene

  • GC02M209328
  • GC02M209777
  • GC02M211016
  • GC02M211255
  • GC02M210878
  • GC02M210760
  • GC02M202897
  • GC02M211052

Summaries for ACADL Gene

Entrez Gene Summary for ACADL Gene

  • The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008]

GeneCards Summary for ACADL Gene

ACADL (Acyl-CoA Dehydrogenase, Long Chain) is a Protein Coding gene. Diseases associated with ACADL include Lcad Deficiency and Hypoglycemia. Among its related pathways are Mitochondrial Fatty Acid Beta-Oxidation and Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha). GO annotations related to this gene include flavin adenine dinucleotide binding and fatty-acyl-CoA binding. An important paralog of this gene is ACAD8.

No data available for UniProtKB/Swiss-Prot , Tocris Summary , Gene Wiki entry , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for ACADL Gene

Genomics for ACADL Gene

Regulatory Elements for ACADL Gene

Enhancers for ACADL Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around ACADL on UCSC Golden Path with GeneCards custom track

Genomic Location for ACADL Gene

210,187,939 bp from pter
210,225,491 bp from pter
37,553 bases
Minus strand

Genomic View for ACADL Gene

Genes around ACADL on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
ACADL Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for ACADL Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for ACADL Gene

Proteins for ACADL Gene

  • Protein details for ACADL Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Long-chain specific acyl-CoA dehydrogenase, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • B2R8T3
    • Q8IUN8

    Protein attributes for ACADL Gene

    430 amino acids
    Molecular mass:
    47656 Da
    Name=FAD; Xref=ChEBI:CHEBI:57692;
    Quaternary structure:
    • Homotetramer.
    • A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.

neXtProt entry for ACADL Gene

Proteomics data for ACADL Gene at MOPED

Selected DME Specific Peptides for ACADL Gene

Post-translational modifications for ACADL Gene

  • Acetylation at Lys-318 and Lys-322 in proximity of the cofactor-binding sites strongly reduces catalytic activity. These sites are deacetylated by SIRT3 (By similarity).
  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for ACADL Gene

ENSEMBL proteins:
REFSEQ proteins:

Domains & Families for ACADL Gene

Gene Families for ACADL Gene

Suggested Antigen Peptide Sequences for ACADL Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the acyl-CoA dehydrogenase family.
  • Belongs to the acyl-CoA dehydrogenase family.
genes like me logo Genes that share domains with ACADL: view

Function for ACADL Gene

Molecular function for ACADL Gene

GENATLAS Biochemistry:
acyl-CoA dehydrogenase,long chain (C10-C18),mitochondrial,fatty acid beta-oxidation
UniProtKB/Swiss-Prot CatalyticActivity:
Long-chain-acyl-CoA + electron-transfer flavoprotein = long-chain-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein.

Enzyme Numbers (IUBMB) for ACADL Gene

Gene Ontology (GO) - Molecular Function for ACADL Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000062 fatty-acyl-CoA binding IBA --
GO:0004466 long-chain-acyl-CoA dehydrogenase activity IEA,ISS --
GO:0009055 electron carrier activity IBA --
genes like me logo Genes that share ontologies with ACADL: view
genes like me logo Genes that share phenotypes with ACADL: view

Animal Models for ACADL Gene

MGI Knock Outs for ACADL:

Animal Model Products

No data available for Human Phenotype Ontology , Transcription Factor Targets and HOMER Transcription for ACADL Gene

Localization for ACADL Gene

Subcellular locations from UniProtKB/Swiss-Prot for ACADL Gene

Mitochondrion matrix.

Subcellular locations from

Jensen Localization Image for ACADL Gene COMPARTMENTS Subcellular localization image for ACADL gene
Compartment Confidence
mitochondrion 5
cytosol 3
nucleus 1
peroxisome 1

No data available for Gene Ontology (GO) - Cellular Components for ACADL Gene

Pathways & Interactions for ACADL Gene

genes like me logo Genes that share pathways with ACADL: view

UniProtKB/Swiss-Prot P28330-ACADL_HUMAN

  • Pathway: Lipid metabolism; mitochondrial fatty acid beta-oxidation.

Interacting Proteins for ACADL Gene

Gene Ontology (GO) - Biological Process for ACADL Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0001659 temperature homeostasis ISS --
GO:0008152 metabolic process IEA --
GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase IEA,ISS --
GO:0042413 carnitine catabolic process ISS --
GO:0042758 long-chain fatty acid catabolic process IEA,IBA --
genes like me logo Genes that share ontologies with ACADL: view

No data available for SIGNOR curated interactions for ACADL Gene

Drugs & Compounds for ACADL Gene

(6) Drugs for ACADL Gene - From: HMDB and Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
FAD Approved Pharma 0
myristoyl-coa Experimental Pharma 0
Propionyl-CoA Experimental Pharma 0

(36) Additional Compounds for ACADL Gene - From: HMDB and Novoseek

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
  • (E)-S-2-decenoate
  • (E)-S-2-decenoate CoA
  • (E)-S-2-decenoate Coenzyme A
  • (E)-S-2-decenoic acid
  • 2-trans-Decenoyl-CoA
  • (2E)-Dodec-2-enoyl-CoA
  • (2E)-Dodec-2-enoyl-Coenzyme A
  • 2-trans-Dodecenoyl-CoA
  • 2-trans-Dodecenoyl-Coenzyme A
  • (2E)-Hexadecenoyl-CoA
  • (2E)-Hexadecenoyl-Coenzyme A
  • trans-2-Hexadecenoyl-CoA
  • trans-2-Hexadecenoyl-Coenzyme A
  • (E)-S-2-octenoate
  • (E)-S-2-octenoate CoA
  • (E)-S-2-octenoate Coenzyme A
  • (E)-S-2-octenoic acid
  • 2,3-trans-Octenoyl coenzyme A
  • (2E)-Tetradecenoyl-CoA
  • (2E)-Tetradecenoyl-Coenzyme A
  • Trans-tetra-dec-2-enoyl-CoA
  • Trans-tetra-dec-2-enoyl-CoA.
  • Trans-tetra-dec-2-enoyl-Coenzyme A
genes like me logo Genes that share compounds with ACADL: view

Transcripts for ACADL Gene

Unigene Clusters for ACADL Gene

Acyl-CoA dehydrogenase, long chain:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for ACADL Gene

No ASD Table

Relevant External Links for ACADL Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for ACADL Gene

mRNA expression in normal human tissues for ACADL Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for ACADL Gene

This gene is overexpressed in Artery - Tibial (x5.8), Pancreas (x4.8), Thyroid (x4.2), Nerve - Tibial (x4.1), and Artery - Aorta (x4.0).

Protein differential expression in normal tissues from HIPED for ACADL Gene

This gene is overexpressed in Liver (13.5), Islet of Langerhans (10.4), Gallbladder (9.8), Kidney (9.3), and Lung (7.0).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MOPED, and MaxQB for ACADL Gene

SOURCE GeneReport for Unigene cluster for ACADL Gene Hs.471277

genes like me logo Genes that share expression patterns with ACADL: view

Primer Products

No data available for mRNA Expression by UniProt/SwissProt and Protein tissue co-expression partners for ACADL Gene

Orthologs for ACADL Gene

This gene was present in the common ancestor of animals.

Orthologs for ACADL Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia ACADL 35
  • 86.74 (n)
  • 83.49 (a)
  • 83 (a)
(Canis familiaris)
Mammalia ACADL 35
  • 86.74 (n)
  • 84.65 (a)
  • 83 (a)
(Mus musculus)
Mammalia Acadl 35
  • 83.84 (n)
  • 86.95 (a)
Acadl 16
Acadl 36
  • 87 (a)
(Pan troglodytes)
Mammalia ACADL 35
  • 99.77 (n)
  • 99.77 (a)
  • 100 (a)
(Rattus norvegicus)
Mammalia Acadl 35
  • 83.92 (n)
  • 85.55 (a)
(Monodelphis domestica)
Mammalia ACADL 36
  • 83 (a)
(Ornithorhynchus anatinus)
Mammalia ACADL 36
  • 85 (a)
(Gallus gallus)
Aves ACADL 35
  • 73.83 (n)
  • 78.04 (a)
  • 77 (a)
(Anolis carolinensis)
Reptilia ACADL 36
  • 78 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia acadl 35
  • 72.47 (n)
  • 79.8 (a)
Str.8318 35
African clawed frog
(Xenopus laevis)
Amphibia Xl.5962 35
(Danio rerio)
Actinopterygii acadl 35
  • 68.6 (n)
  • 76.26 (a)
zgc55656 35
acadl 36
  • 70 (a)
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.10227 35
(Caenorhabditis elegans)
Secernentea C02D5.1 37
  • 31 (a)
C37A2.3 37
  • 32 (a)
acdh-6 36
  • 29 (a)
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4861 35
sea squirt
(Ciona savignyi)
Ascidiacea CSA.2732 36
  • 64 (a)
Species with no ortholog for ACADL:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • fruit fly (Drosophila melanogaster)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for ACADL Gene

Gene Tree for ACADL (if available)
Gene Tree for ACADL (if available)

Paralogs for ACADL Gene

Paralogs for ACADL Gene

(7) SIMAP similar genes for ACADL Gene using alignment to 1 proteins:

genes like me logo Genes that share paralogs with ACADL: view

Variants for ACADL Gene

Sequence variations from dbSNP and Humsavar for ACADL Gene

SNP ID Clin Chr 02 pos Sequence Context AA Info Type
rs1801204 - 210,203,407(-) AGCTA(C/G)TGAAT reference, missense
rs2286963 - 210,195,326(+) TAATT(G/T)ATGTT reference, missense
rs1848709 -- 210,224,092(-) aggca(C/T)gcacc intron-variant
rs1858153 -- 210,188,619(-) TCGCT(A/C)TAGTA utr-variant-3-prime
rs2058508 -- 210,197,621(+) CACTT(C/T)ATTTG intron-variant

Variation tolerance for ACADL Gene

Residual Variation Intolerance Score: 65.4% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 8.43; 85.64% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for ACADL Gene

Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot and Structural Variations from Database of Genomic Variants (DGV) for ACADL Gene

Disorders for ACADL Gene

MalaCards: The human disease database

(8) MalaCards diseases for ACADL Gene - From: Orphanet, DISEASES, Novoseek, and GeneCards

Disorder Aliases PubMed IDs
lcad deficiency
  • acadl deficiency
  • hypoglycaemia
acyl-coa dehydrogenase, medium chain, deficiency of
  • acyl-coa dehydrogenase medium-chain deficiency
holocarboxylase synthetase deficiency
  • biotin- ligase deficiency
surfactant dysfunction
  • pulmonary surfactant metabolism dysfunction
- elite association - COSMIC cancer census association via MalaCards
Search ACADL in MalaCards View complete list of genes associated with diseases


  • Acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]: An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Note=The disease is caused by mutations affecting the gene represented in this entry.

Genatlas disease for ACADL Gene

dicarboxylic aciduria,non ketotic hypoglycemia ,Reye-like syndrome,sudden infant death syndrome

Relevant External Links for ACADL

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
genes like me logo Genes that share disorders with ACADL: view

Publications for ACADL Gene

  1. Molecular cloning and nucleotide sequence of cDNAs encoding human long-chain acyl-CoA dehydrogenase and assignment of the location of its gene (ACADL) to chromosome 2. (PMID: 1774065) Indo Y. … Tanaka K. (Genomics 1991) 2 3 4 23 67
  2. Low expression of long-chain acyl-CoA dehydrogenase in human skeletal muscle. (PMID: 20363655) Maher A.C. … Tarnopolsky M.A. (Mol. Genet. Metab. 2010) 3 23
  3. Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids. (PMID: 10832093) Lea W. … Schulz H. (Biochim. Biophys. Acta 2000) 3 23
  4. 2,6-Dimethylheptanoyl-CoA is a specific substrate for long-chain acyl-CoA dehydrogenase (LCAD): evidence for a major role of LCAD in branched-chain fatty acid oxidation. (PMID: 9714723) Wanders R.J. … Dacremont G. (Biochim. Biophys. Acta 1998) 3 23
  5. Regulation of the human long chain acyl-CoA dehydrogenase gene by nuclear hormone receptor transcription factors. (PMID: 9003458) Zhang Z. … Strauss A.W. (Biochim. Biophys. Acta 1997) 3 23

Products for ACADL Gene

Sources for ACADL Gene