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Aliases for ACOT2 Gene

Aliases for ACOT2 Gene

  • Acyl-CoA Thioesterase 2 2 3 4 5
  • Acyl-Coenzyme A Thioester Hydrolase 2a 3 4
  • Mitochondrial Acyl-CoA Thioesterase 1 2 3
  • Long-Chain Acyl-CoA Thioesterase 2 3 4
  • EC 4 56
  • CTE-IA 3 4
  • ZAP128 3 4
  • PTE2A 3 4
  • PTE2 3 4
  • Peroxisomal Long-Chain Acyl-CoA Thioesterase 2 3
  • Acyl-Coenzyme A Thioesterase 2, Mitochondrial 3
  • CTE1A 3
  • MTE1 3

External Ids for ACOT2 Gene

Previous GeneCards Identifiers for ACOT2 Gene

  • GC14P073106
  • GC14P074036
  • GC14P054203

Summaries for ACOT2 Gene

Entrez Gene Summary for ACOT2 Gene

  • This gene encodes a member of the acyl-CoA thioesterase protein family, and is one of four acyl-CoA hydrolase genes located in a cluster on chromosome 14. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2012]

GeneCards Summary for ACOT2 Gene

ACOT2 (Acyl-CoA Thioesterase 2) is a Protein Coding gene. Diseases associated with ACOT2 include Early-Onset Familial Alzheimer Disease. Among its related pathways are Fatty Acyl-CoA Biosynthesis and Fatty acid elongation. Gene Ontology (GO) annotations related to this gene include receptor binding and palmitoyl-CoA hydrolase activity. An important paralog of this gene is ACOT1.

UniProtKB/Swiss-Prot for ACOT2 Gene

  • Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH. Displays high levels of activity on medium- and long chain acyl CoAs.

Gene Wiki entry for ACOT2 Gene

Additional gene information for ACOT2 Gene

No data available for CIViC summary , Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for ACOT2 Gene

Genomics for ACOT2 Gene

GeneHancer (GH) Regulatory Elements for ACOT2 Gene

Promoters and enhancers for ACOT2 Gene
GeneHancer (GH) Identifier GH Type GH
GH Sources Gene Association Score Total Score TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor
Binding Sites
Gene Targets
GH14I073567 Promoter/Enhancer 2 EPDnew Ensembl ENCODE 556 +1.3 1337 3.8 HDGF PKNOX1 SMAD1 MLX ARID4B SIN3A DMAP1 YBX1 ZNF2 YY1 ACOT2 GC14M073564 GC14M073568 ACOT1 ENSG00000258695 DCAF4 LOC100506476 DNAL1
GH14I073566 Enhancer 0.4 Ensembl 571.7 -0.9 -919 0.6 NFYC ZBTB8A ACOT2 GC14M073564 ENSG00000258695 LOC100506476 HEATR4
GH14I073536 Promoter/Enhancer 2.1 EPDnew Ensembl ENCODE 7.1 -29.5 -29521 3.5 ARID4B SIN3A DMAP1 ZNF48 YY1 SP5 MIER2 REST KAT8 NFIL3 ACOT1 GC14M073540 NT5CP2 ACOT2 BBOF1 ENSG00000258695 HEATR4
GH14I072891 Promoter/Enhancer 2.1 EPDnew Ensembl ENCODE dbSUPER 7 -674.0 -674022 3.9 PKNOX1 ATF1 ARID4B SIN3A ZNF2 YY1 GLIS2 ATF7 FOS RXRA DPF3 DCAF4 PSEN1 RBM25 ZNF410 ELMSAN1 ACOT2 LOC101928123 GC14M072854 ENSG00000206751
GH14I072780 Enhancer 1.2 Ensembl ENCODE dbSUPER 4.7 -786.1 -786051 2.3 ELF3 SOX13 FOXA2 JUN ZNF384 BATF RARA ETS1 FOSL2 NR2F6 DPF3 ACOT2 GC14M072730 GC14M072854 ENSG00000206751
- Elite GeneHancer and/or Elite GeneHancer-gene association Download GeneHancer data dump

GeneHancers around ACOT2 on UCSC Golden Path with GeneCards custom track

Top Transcription factor binding sites by QIAGEN in the ACOT2 gene promoter:

Genomic Locations for ACOT2 Gene

Genomic Locations for ACOT2 Gene
8,039 bases
Plus strand

Genomic View for ACOT2 Gene

Genes around ACOT2 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
ACOT2 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for ACOT2 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for ACOT2 Gene

Proteins for ACOT2 Gene

  • Protein details for ACOT2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Acyl-coenzyme A thioesterase 2, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • Q3I5F8
    • Q53EK4
    • Q9NUX4

    Protein attributes for ACOT2 Gene

    483 amino acids
    Molecular mass:
    53218 Da
    Quaternary structure:
    • Monomer.
    • Sequence=AAC42007.1; Type=Frameshift; Positions=215, 226; Evidence={ECO:0000305};

    Three dimensional structures from OCA and Proteopedia for ACOT2 Gene

    Alternative splice isoforms for ACOT2 Gene


neXtProt entry for ACOT2 Gene

Selected DME Specific Peptides for ACOT2 Gene


Post-translational modifications for ACOT2 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for ACOT2 Gene

Domains & Families for ACOT2 Gene

Gene Families for ACOT2 Gene

Human Protein Atlas (HPA):
  • Enzymes
  • Plasma proteins
  • Predicted intracellular proteins

Suggested Antigen Peptide Sequences for ACOT2 Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the C/M/P thioester hydrolase family.
  • Belongs to the C/M/P thioester hydrolase family.
genes like me logo Genes that share domains with ACOT2: view

Function for ACOT2 Gene

Molecular function for ACOT2 Gene

UniProtKB/Swiss-Prot BiophysicochemicalProperties:
Kinetic parameters: KM=40.3 uM for C10-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=8.9 uM for C12-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=1.6 uM for C14-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=2.0 uM for C16-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=2.8 uM for C18-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=4.8 uM for C20-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=4.5 uM for C16:1-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=6.1 uM for C18:1-acyl-CoA {ECO:0000269 PubMed:16940157}; KM=4.3 uM for C18:1-trans-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=212 nmol/min/mg enzyme toward C10-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=681 nmol/min/mg enzyme toward C12-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=766 nmol/min/mg enzyme toward C14-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=656 nmol/min/mg enzyme toward C16-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=488 nmol/min/mg enzyme toward C18-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=408 nmol/min/mg enzyme toward C20-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=661 nmol/min/mg enzyme toward C16:1-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=304 nmol/min/mg enzyme toward C18:1-acyl-CoA {ECO:0000269 PubMed:16940157}; Vmax=418 nmol/min/mg enzyme toward C18:1-trans-acyl-CoA {ECO:0000269 PubMed:16940157};
UniProtKB/Swiss-Prot CatalyticActivity:
Palmitoyl-CoA + H(2)O = CoA + palmitate.
UniProtKB/Swiss-Prot Function:
Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH. Displays high levels of activity on medium- and long chain acyl CoAs.

Enzyme Numbers (IUBMB) for ACOT2 Gene

Phenotypes From GWAS Catalog for ACOT2 Gene

Gene Ontology (GO) - Molecular Function for ACOT2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005102 receptor binding IPI 20178365
GO:0016290 palmitoyl-CoA hydrolase activity IEA --
GO:0016787 hydrolase activity IEA --
GO:0016790 thiolester hydrolase activity IEA --
GO:0047617 acyl-CoA hydrolase activity TAS,IDA --
genes like me logo Genes that share ontologies with ACOT2: view
genes like me logo Genes that share phenotypes with ACOT2: view

Animal Model Products

CRISPR Products

miRNA for ACOT2 Gene

Inhibitory RNA Products

No data available for Human Phenotype Ontology , Animal Models , Transcription Factor Targets and HOMER Transcription for ACOT2 Gene

Localization for ACOT2 Gene

Subcellular locations from UniProtKB/Swiss-Prot for ACOT2 Gene


Subcellular locations from

Extracellular space Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi Apparatus Nucleus Mitochondrion 0 1 2 3 4 5 Confidence
COMPARTMENTS Subcellular localization image for ACOT2 gene
Compartment Confidence
extracellular 5
mitochondrion 5
peroxisome 3
plasma membrane 1
nucleus 1
cytosol 1
golgi apparatus 1

Subcellular locations from the

Human Protein Atlas (HPA)

Gene Ontology (GO) - Cellular Components for ACOT2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA 16940157
GO:0005759 mitochondrial matrix TAS --
GO:0070062 extracellular exosome IDA,HDA 23376485
genes like me logo Genes that share ontologies with ACOT2: view

Pathways & Interactions for ACOT2 Gene

genes like me logo Genes that share pathways with ACOT2: view

Gene Ontology (GO) - Biological Process for ACOT2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000038 very long-chain fatty acid metabolic process IDA 16940157
GO:0001676 long-chain fatty acid metabolic process IDA 16940157
GO:0006637 acyl-CoA metabolic process TAS,IEA --
genes like me logo Genes that share ontologies with ACOT2: view

No data available for SIGNOR curated interactions for ACOT2 Gene

Drugs & Compounds for ACOT2 Gene

(13) Drugs for ACOT2 Gene - From: HMDB

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Docosahexaenoic acid Approved, Investigational Pharma Full agonist, Agonist 0
oleic acid Approved, Investigational, Vet_approved Pharma Full agonist, Agonist 0
Palmitic Acid Approved, Experimental Pharma Full agonist, Agonist 23
Stearic acid Approved, Experimental Pharma 0
Water Approved Pharma 0

(42) Additional Compounds for ACOT2 Gene - From: HMDB

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 ACOT2: view

Transcripts for ACOT2 Gene

Unigene Clusters for ACOT2 Gene

Acyl-CoA thioesterase 2:
Representative Sequences:

CRISPR Products

Inhibitory RNA Products

Alternative Splicing Database (ASD) splice patterns (SP) for ACOT2 Gene

No ASD Table

Relevant External Links for ACOT2 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for ACOT2 Gene

mRNA expression in normal human tissues from GTEx, Illumina, BioGPS, and CGAP SAGE for ACOT2 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Protein differential expression in normal tissues from HIPED for ACOT2 Gene

This gene is overexpressed in Kidney (10.8), Prostate (8.5), and Heart (7.4).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MaxQB, and MOPED for ACOT2 Gene

NURSA nuclear receptor signaling pathways regulating expression of ACOT2 Gene:


SOURCE GeneReport for Unigene cluster for ACOT2 Gene:


mRNA Expression by UniProt/SwissProt for ACOT2 Gene:

Tissue specificity: Strongest expression in heart, liver, muscle and kidney. Weak in placenta and pancreas.

Evidence on tissue expression from TISSUES for ACOT2 Gene

  • Nervous system(4.7)
  • Lung(4.5)
  • Spleen(4.2)
  • Liver(2.3)
  • Heart(2)
  • Muscle(2)
genes like me logo Genes that share expression patterns with ACOT2: view

No data available for mRNA differential expression in normal tissues , Protein tissue co-expression partners and Phenotype-based relationships between genes and organs from Gene ORGANizer for ACOT2 Gene

Orthologs for ACOT2 Gene

This gene was present in the common ancestor of animals.

Orthologs for ACOT2 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia ACOT2 33
  • 98.62 (n)
(Bos Taurus)
Mammalia ACOT2 33 34
  • 85.67 (n)
(Canis familiaris)
Mammalia ACOT2 33
  • 84.76 (n)
-- 34
  • 80 (a)
(Mus musculus)
Mammalia Acot2 33 16 34
  • 79.81 (n)
Acot1 34
  • 77 (a)
Acot3 34
  • 75 (a)
Acot5 34
  • 74 (a)
(Rattus norvegicus)
Mammalia Acot2 33
  • 79.48 (n)
(Ornithorhynchus anatinus)
Mammalia -- 34
  • 66 (a)
-- 34
  • 60 (a)
(Monodelphis domestica)
Mammalia -- 34
  • 60 (a)
(Gallus gallus)
Aves LOC771753 33
  • 67.32 (n)
-- 34
  • 62 (a)
(Anolis carolinensis)
Reptilia -- 34
  • 62 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia acot2 33
  • 60.69 (n)
Str.12247 33
(Danio rerio)
Actinopterygii zgc:77651 34
  • 33 (a)
(Caenorhabditis elegans)
Secernentea K05B2.4 34
  • 28 (a)
W03D8.8 34
  • 27 (a)
C31H5.6 34
  • 25 (a)
T05E7.1 34
  • 22 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 34
  • 39 (a)
-- 34
  • 35 (a)
-- 34
  • 35 (a)
-- 34
  • 35 (a)
-- 34
  • 34 (a)
-- 34
  • 33 (a)
-- 34
  • 31 (a)
-- 34
  • 30 (a)
-- 34
  • 28 (a)
-- 34
  • 28 (a)
Species where no ortholog for ACOT2 was found in the sources mined by GeneCards:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • 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)
  • rainbow trout (Oncorhynchus mykiss)
  • 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 ACOT2 Gene

Gene Tree for ACOT2 (if available)
Gene Tree for ACOT2 (if available)

Paralogs for ACOT2 Gene

Paralogs for ACOT2 Gene

(4) SIMAP similar genes for ACOT2 Gene using alignment to 2 proteins:

genes like me logo Genes that share paralogs with ACOT2: view

Variants for ACOT2 Gene

Sequence variations from dbSNP and Humsavar for ACOT2 Gene

SNP ID Clin Chr 14 pos Variation AA Info Type
rs1000463619 -- 73,567,460(+) A/G upstream_transcript_variant
rs1000800252 -- 73,566,451(+) G/C upstream_transcript_variant
rs1000895422 -- 73,566,286(+) A/G upstream_transcript_variant
rs1001015638 -- 73,571,313(+) T/C intron_variant
rs1001132847 -- 73,571,681(+) C/T intron_variant

Structural Variations from Database of Genomic Variants (DGV) for ACOT2 Gene

Variant ID Type Subtype PubMed ID
dgv1935n100 CNV gain 25217958
dgv3764n54 CNV gain 21841781
dgv3774n54 CNV loss 21841781
dgv408e214 CNV gain 21293372
dgv656e212 CNV gain 25503493
dgv662e212 CNV loss 25503493
esv1006342 CNV deletion 20482838
esv2672436 CNV deletion 23128226
esv2748867 CNV deletion 23290073
esv2759996 CNV gain 17122850
esv2760335 CNV gain+loss 21179565
esv2761566 CNV gain+loss 21179565
esv29080 CNV gain+loss 19812545
esv29945 CNV loss 18421352
esv30006 CNV gain 17803354
esv33155 CNV gain+loss 17666407
esv3634913 CNV loss 21293372
esv3892561 CNV gain 25118596
esv3892562 CNV gain 25118596
nsv1035460 CNV gain 25217958
nsv1053281 CNV gain 25217958
nsv1350 CNV deletion 18451855
nsv428295 CNV gain 18775914
nsv436178 CNV deletion 17901297
nsv442346 CNV gain+loss 18776908
nsv510643 CNV deletion 20534489
nsv514751 CNV gain 21397061
nsv565162 CNV loss 21841781
nsv565172 CNV loss 21841781
nsv819954 CNV gain 19587683
nsv821106 CNV deletion 20802225
nsv826976 CNV loss 20364138
nsv826985 CNV loss 20364138
nsv826987 CNV loss 20364138
nsv826988 CNV loss 20364138
nsv832828 CNV gain+loss 17160897
nsv9153 CNV gain+loss 18304495

Variation tolerance for ACOT2 Gene

Residual Variation Intolerance Score: 82.1% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 3.71; 57.47% of all genes are more intolerant (likely to be disease-causing)

Additional Variant Information for ACOT2 Gene

SNPedia medical, phenotypic, and genealogical associations of SNPs for

SNP Genotyping and Copy Number Assay Products

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for ACOT2 Gene

Disorders for ACOT2 Gene

MalaCards: The human disease database

(1) MalaCards diseases for ACOT2 Gene - From: GeneCards

Disorder Aliases PubMed IDs
early-onset familial alzheimer disease
  • eofad
- elite association - COSMIC cancer census association via MalaCards
Search ACOT2 in MalaCards View complete list of genes associated with diseases

Additional Disease Information for ACOT2

Genetic Association Database
Human Genome Epidemiology Navigator
ATLAS of Genetics and Cytogenetics in Oncology and Haematology
genes like me logo Genes that share disorders with ACOT2: view

No data available for UniProtKB/Swiss-Prot and Genatlas for ACOT2 Gene

Publications for ACOT2 Gene

  1. Crystal structure of human mitochondrial acyl-CoA thioesterase (ACOT2). (PMID: 19497300) Mandel CR … Tong L (Biochemical and biophysical research communications 2009) 3 4 22 58
  2. Analysis of the mouse and human acyl-CoA thioesterase (ACOT) gene clusters shows that convergent, functional evolution results in a reduced number of human peroxisomal ACOTs. (PMID: 16940157) Hunt MC … Alexson SE (FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2006) 2 3 4 58
  3. Identification of PTE2, a human peroxisomal long-chain acyl-CoA thioesterase. (PMID: 10944470) Jones JM … Gould SJ (Biochemical and biophysical research communications 2000) 3 4 22 58
  4. Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score. (PMID: 20379614) Rose JE … Uhl GR (Molecular medicine (Cambridge, Mass.) 2010) 3 44 58
  5. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PMID: 20877624) Hendrickson SL … O'Brien SJ (PloS one 2010) 3 44 58

Products for ACOT2 Gene

Sources for ACOT2 Gene

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