Free for academic non-profit institutions. Other users need a Commercial license

Aliases for ACADL Gene

Aliases for ACADL Gene

  • Acyl-CoA Dehydrogenase, Long Chain 2 3
  • LCAD 3 4 6
  • Acyl-Coenzyme A Dehydrogenase, Long Chain 2 3
  • Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial 3
  • EC 64
  • EC 4
  • EC 1.3.99 64
  • ACAD4 3

External Ids for ACADL Gene

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 fatty acid oxidation disorders. Among its related pathways are Metabolism and Metabolism. GO annotations related to this gene include flavin adenine dinucleotide binding and acyl-CoA dehydrogenase activity. 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

Genomic Location for ACADL Gene

210,187,908 bp from pter
210,225,491 bp from pter
37,584 bases
Minus strand

Genomic View for ACADL Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for ACADL Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for ACADL Gene

Regulatory Elements 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:
Reactome Protein details:
REFSEQ proteins:

Domains for ACADL Gene


  • Belongs to the acyl-CoA dehydrogenase family.:
    • P28330
genes like me logo Genes that share domains with ACADL: view

No data available for Gene Families for ACADL Gene

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 IEA --
GO:0003995 acyl-CoA dehydrogenase activity TAS --
GO:0004466 long-chain-acyl-CoA dehydrogenase activity ISS --
GO:0016401 palmitoyl-CoA oxidase activity ISS --
GO:0016627 oxidoreductase activity, acting on the CH-CH group of donors --
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:

miRNA for ACADL Gene

miRTarBase miRNAs that target ACADL

No data available for Transcription Factor Targeting 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
peroxisome 2
nucleus 1

Gene Ontology (GO) - Cellular Components for ACADL Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005737 cytoplasm IDA --
GO:0005739 mitochondrion IDA --
GO:0005759 mitochondrial matrix TAS --
GO:0031966 mitochondrial membrane IEA --
genes like me logo Genes that share ontologies with ACADL: view

Pathways 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:0006635 fatty acid beta-oxidation TAS --
GO:0008152 metabolic process --
GO:0019254 carnitine metabolic process, CoA-linked ISS --
GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase ISS --
genes like me logo Genes that share ontologies with ACADL: view

Compounds for ACADL Gene

(35) HMDB Compounds for ACADL Gene

Compound Synonyms Cas Number PubMed IDs
  • (E)-S-2-decenoate Coenzyme A
  • 2-trans-Dodecenoyl-Coenzyme A
  • trans-2-Hexadecenoyl-CoA
  • (E)-S-2-octenoate Coenzyme A
  • (2E)-Tetradecenoyl-Coenzyme A

(6) Novoseek inferred chemical compound relationships for ACADL Gene

Compound -log(P) Hits PubMed IDs
acyl-coa 91 37
pristanic acid 78.1 2
fatty acid 71.1 12
carnitine 66.4 4
lipid 15.6 1
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 (5.8), Pancreas (4.8), Thyroid (4.2), Nerve - Tibial (4.1), and Artery - Aorta (4.0).

Integrated Proteomics: protein expression 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 expressions with ACADL: view

Orthologs for ACADL Gene

This gene was present in the common ancestor of animals.

Orthologs for ACADL Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia ACADL 36
  • 99.77 (n)
  • 99.77 (a)
  • 100 (a)
(Bos Taurus)
Mammalia ACADL 36
  • 86.74 (n)
  • 83.49 (a)
  • 83 (a)
(Canis familiaris)
Mammalia ACADL 36
  • 86.74 (n)
  • 84.65 (a)
  • 83 (a)
(Mus musculus)
Mammalia Acadl 36
  • 83.84 (n)
  • 86.95 (a)
Acadl 16
Acadl 37
  • 87 (a)
(Monodelphis domestica)
Mammalia ACADL 37
  • 83 (a)
(Ornithorhynchus anatinus)
Mammalia ACADL 37
  • 85 (a)
(Rattus norvegicus)
Mammalia Acadl 36
  • 83.92 (n)
  • 85.55 (a)
(Gallus gallus)
Aves ACADL 36
  • 73.83 (n)
  • 78.04 (a)
  • 77 (a)
(Anolis carolinensis)
Reptilia ACADL 37
  • 78 (a)
African clawed frog
(Xenopus laevis)
Amphibia Xl.5962 36
tropical clawed frog
(Silurana tropicalis)
Amphibia acadl 36
  • 72.47 (n)
  • 79.8 (a)
Str.8318 36
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.10227 36
(Danio rerio)
Actinopterygii acadl 36
  • 68.6 (n)
  • 76.26 (a)
acadl 37
  • 70 (a)
zgc55656 36
(Caenorhabditis elegans)
Secernentea acdh-6 37
  • 29 (a)
C02D5.1 38
  • 31 (a)
C37A2.3 38
  • 32 (a)
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4861 36
sea squirt
(Ciona savignyi)
Ascidiacea CSA.2732 37
  • 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

Selected 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 MAF
rs263678 -- 210,202,879(+) cctcc(A/G)cctcc intron-variant
rs263679 -- 210,202,764(+) tcttc(C/G)cttac intron-variant
rs263683 -- 210,194,775(+) TTATA(C/G)TTATT intron-variant
rs263684 -- 210,187,564(+) ccaac(A/G)tggcg downstream-variant-500B
rs1155845 -- 210,189,875(-) TTGTT(A/G)GTGAC intron-variant

Relevant External Links for ACADL Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Structural Variations from Database of Genomic Variants (DGV) for ACADL Gene

Disorders for ACADL Gene


  • 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.

(5) Novoseek inferred disease relationships for ACADL Gene

Disease -log(P) Hits PubMed IDs
mcad deficiency 73.2 1
enzyme deficiency 63.9 3
cardiac hypertrophy 47.7 2
hypoglycemia 46.1 3
death sudden 23.2 1

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
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
  2. Immunochemical characterization of variant long-chain acyl-CoA dehydrogenase in cultured fibroblasts from nine patients with long-chain acyl-CoA dehydrogenase deficiency. (PMID: 1945557) Indo Y. … Tanaka K. (Pediatr. Res. 1991) 3 23
  3. 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
  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. 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

Products for ACADL Gene

  • Addgene plasmids for ACADL

Sources for ACADL Gene

Back to Top