Gene/Proteome Database (LMPD)

LMPD ID
LMP000585
Gene ID
30
Species
Homo sapiens (Human)
Gene Name
acetyl-CoA acyltransferase 1
Gene Symbol
ACAA1
Synonyms
ACAA; PTHIO; THIO
Alternate Names
3-ketoacyl-CoA thiolase, peroxisomal; beta-ketothiolase; peroxisomal 3-oxoacyl-CoA thiolase; acetyl-Coenzyme A acyltransferase 1; peroxisomal 3-oxoacyl-Coenzyme A thiolase
Chromosome
3
Map Location
3p22.2
EC Number
2.3.1.16
Summary
This gene encodes an enzyme operative in the beta-oxidation system of the peroxisomes. Deficiency of this enzyme leads to pseudo-Zellweger syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for ACAA1

Proteins

3-ketoacyl-CoA thiolase, peroxisomal isoform a
Refseq ID NP_001598
Protein GI 4501853
UniProt ID P09110
mRNA ID NM_001607
Length 424
RefSeq Status REVIEWED
MQRLQVVLGHLRGPADSGWMPQAAPCLSGAPQASAADVVVVHGRRTAICRAGRGGFKDTTPDELLSAVMTAVLKDVNLRPEQLGDICVGNVLQPGAGAIMARIAQFLSDIPETVPLSTVNRQCSSGLQAVASIAGGIRNGSYDIGMACGVESMSLADRGNPGNITSRLMEKEKARDCLIPMGITSENVAERFGISREKQDTFALASQQKAARAQSKGCFQAEIVPVTTTVHDDKGTKRSITVTQDEGIRPSTTMEGLAKLKPAFKKDGSTTAGNSSQVSDGAAAILLARRSKAEELGLPILGVLRSYAVVGVPPDIMGIGPAYAIPVALQKAGLTVSDVDIFEINEAFASQAAYCVEKLRLPPEKVNPLGGAVALGHPLGCTGARQVITLLNELKRRGKRAYGVVSMCIGTGMGAAAVFEYPGN
transit_peptide: 1..26 experiment: experimental evidence, no additional details recorded note: Peroxisome; propagated from UniProtKB/Swiss-Prot (P09110.2) calculated_mol_wt: 2819 peptide sequence: MQRLQVVLGHLRGPADSGWMPQAAPC mat_peptide: 27..424 product: 3-ketoacyl-CoA thiolase, peroxisomal experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P09110.2) calculated_mol_wt: 41491 peptide sequence: LSGAPQASAADVVVVHGRRTAICRAGRGGFKDTTPDELLSAVMTAVLKDVNLRPEQLGDICVGNVLQPGAGAIMARIAQFLSDIPETVPLSTVNRQCSSGLQAVASIAGGIRNGSYDIGMACGVESMSLADRGNPGNITSRLMEKEKARDCLIPMGITSENVAERFGISREKQDTFALASQQKAARAQSKGCFQAEIVPVTTTVHDDKGTKRSITVTQDEGIRPSTTMEGLAKLKPAFKKDGSTTAGNSSQVSDGAAAILLARRSKAEELGLPILGVLRSYAVVGVPPDIMGIGPAYAIPVALQKAGLTVSDVDIFEINEAFASQAAYCVEKLRLPPEKVNPLGGAVALGHPLGCTGARQVITLLNELKRRGKRAYGVVSMCIGTGMGAAAVFEYPGN
3-ketoacyl-CoA thiolase, peroxisomal isoform b
Refseq ID NP_001123882
Protein GI 194353979
UniProt ID P09110
mRNA ID NM_001130410
Length 331
RefSeq Status REVIEWED
MQRLQVVLGHLRGPADSGWMPQAAPCLSGAPQASAADVVVVHGRRTAICRAGRGGFKDTTPDELLSAVMTAVLKDVNLRPEQLGDICVGNVLQPGAGAIMARIAQFLSDIPETVPLSTVNRQCSSGLQAVASIAGGIRNGSYDIGMACGITSENVAERFGISREKQDTFALASQQKAARAQSKGCFQAEIVPVTTTVHDDKGTKRSITVTQDEGIRPSTTMEGLAKLKPAFKKDGSTTAGLTVSDVDIFEINEAFASQAAYCVEKLRLPPEKVNPLGGAVALGHPLGCTGARQVITLLNELKRRGKRAYGVVSMCIGTGMGAAAVFEYPGN

Gene Information

Entrez Gene ID
30
Gene Name
acetyl-CoA acyltransferase 1
Gene Symbol
ACAA1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0043231 IDA:HPA C intracellular membrane-bounded organelle
GO:0016020 IDA:UniProtKB C membrane
GO:0005782 TAS:Reactome C peroxisomal matrix
GO:0005777 IDA:UniProtKB C peroxisome
GO:0003988 IEA:UniProtKB-EC F acetyl-CoA C-acyltransferase activity
GO:0016401 IMP:UniProtKB F palmitoyl-CoA oxidase activity
GO:0036109 TAS:Reactome P alpha-linolenic acid metabolic process
GO:0008206 IMP:UniProtKB P bile acid metabolic process
GO:0044255 TAS:Reactome P cellular lipid metabolic process
GO:0006635 IMP:UniProtKB P fatty acid beta-oxidation
GO:0033540 TAS:Reactome P fatty acid beta-oxidation using acyl-CoA oxidase
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0033559 TAS:Reactome P unsaturated fatty acid metabolic process
GO:0000038 IMP:UniProtKB P very long-chain fatty acid metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa03320 PPAR signaling pathway
hsa04146 Peroxisome

BIOCYC Pathway Links

BIOCYC Pathway ID Description
PWY66-368 ketolysis

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_121147 alpha-linolenic acid (ALA) metabolism

Domain Information

InterPro Annotations

Accession Description
IPR002155 Thiolase
IPR020617 Thiolase, C-terminal
IPR020616 Thiolase, N-terminal
IPR020610 Thiolase, active site
IPR020615 Thiolase, acyl-enzyme intermediate active site
IPR020613 Thiolase, conserved site
IPR016039 Thiolase-like
IPR016038 Thiolase-like, subgroup

UniProt Annotations

Entry Information

Gene Name
acetyl-CoA acyltransferase 1
Protein Entry
THIK_HUMAN
UniProt ID
P09110
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P09110-1; Sequence=Displayed; Name=2; IsoId=P09110-2; Sequence=VSP_046195, VSP_046196; Note=No experimental confirmation available.;
Catalytic Activity Acyl-CoA + acetyl-CoA = CoA + 3-oxoacyl-CoA.
Induction Peroxisomal thiolase is markedly induced (at the level of transcription) by various hypolipidemic compounds in parallel with the other two enzymes of the peroxisomal beta-oxidation system.
Pathway Lipid metabolism; fatty acid metabolism.
Similarity Belongs to the thiolase family.
Subcellular Location Peroxisome.
Subunit Homodimer.

Identical and Related Proteins

Unique RefSeq proteins for LMP000585 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4501853 RefSeq NP_001598 424 3-ketoacyl-CoA thiolase, peroxisomal isoform a
194353979 RefSeq NP_001123882 331 3-ketoacyl-CoA thiolase, peroxisomal isoform b

Identical Sequences to LMP000585 proteins

Reference Database Accession Length Protein Name
GI:194353979 GenBank EAW64516.1 331 acetyl-Coenzyme A acyltransferase 1 (peroxisomal 3-oxoacyl-Coenzyme A thiolase), isoform CRA_d [Homo sapiens]
GI:4501853 GenBank AEF74578.1 424 Sequence 81 from patent US 7939271
GI:4501853 GenBank JAA05628.1 424 acetyl-CoA acyltransferase 1 [Pan troglodytes]
GI:4501853 GenBank JAA15600.1 424 acetyl-CoA acyltransferase 1 [Pan troglodytes]
GI:4501853 GenBank JAA22694.1 424 acetyl-CoA acyltransferase 1 [Pan troglodytes]
GI:4501853 GenBank JAA35503.1 424 acetyl-CoA acyltransferase 1 [Pan troglodytes]
GI:4501853 GenBank AIC48201.1 424 ACAA1, partial [synthetic construct]

Related Sequences to LMP000585 proteins

Reference Database Accession Length Protein Name
GI:4501853 DBBJ BAE72965.1 424 hypothetical protein [Macaca fascicularis]
GI:194353979 GenBank AAH14474.1 331 ACAA1 protein [Homo sapiens]
GI:4501853 GenBank AFE77986.1 424 3-ketoacyl-CoA thiolase, peroxisomal isoform a [Macaca mulatta]
GI:4501853 GenBank AFH32148.1 424 3-ketoacyl-CoA thiolase, peroxisomal isoform a [Macaca mulatta]
GI:194353979 GenBank AIC53940.1 331 ACAA1, partial [synthetic construct]
GI:4501853 RefSeq XP_002813979.1 424 PREDICTED: 3-ketoacyl-CoA thiolase, peroxisomal [Pongo abelii]
GI:194353979 RefSeq XP_003781849.1 331 PREDICTED: 3-ketoacyl-CoA thiolase, peroxisomal isoform 2 [Otolemur garnettii]
GI:4501853 RefSeq XP_004033892.1 424 PREDICTED: 3-ketoacyl-CoA thiolase, peroxisomal isoform 1 [Gorilla gorilla gorilla]
GI:194353979 RefSeq XP_004033893.1 331 PREDICTED: 3-ketoacyl-CoA thiolase, peroxisomal isoform 2 [Gorilla gorilla gorilla]
GI:4501853 RefSeq XP_003256905.2 424 PREDICTED: LOW QUALITY PROTEIN: 3-ketoacyl-CoA thiolase, peroxisomal isoform 2 [Nomascus leucogenys]
GI:194353979 RefSeq XP_005546712.1 361 PREDICTED: 3-ketoacyl-CoA thiolase, peroxisomal isoform X2 [Macaca fascicularis]
GI:194353979 RefSeq XP_006713185.1 364 PREDICTED: 3-ketoacyl-CoA thiolase, peroxisomal isoform X1 [Homo sapiens]