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Protein

3-ketoacyl-CoA thiolase, peroxisomal

Gene

ACAA1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalytic activityi

Acyl-CoA + acetyl-CoA = CoA + 3-oxoacyl-CoA.

Pathwayi: fatty acid metabolism

This protein is involved in the pathway fatty acid metabolism, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway fatty acid metabolism and in Lipid metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei123Acyl-thioester intermediateBy similarity1
Active sitei377Proton acceptorPROSITE-ProRule annotation1
Active sitei408Proton acceptorPROSITE-ProRule annotation1

GO - Molecular functioni

  • acetate CoA-transferase activity Source: Reactome
  • acetyl-CoA C-acyltransferase activity Source: GO_Central
  • palmitoyl-CoA oxidase activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionAcyltransferase, Transferase
Biological processFatty acid metabolism, Lipid metabolism

Enzyme and pathway databases

BioCyciMetaCyc:HS00752-MONOMER
ReactomeiR-HSA-2046106 alpha-linolenic acid (ALA) metabolism
R-HSA-390247 Beta-oxidation of very long chain fatty acids
R-HSA-6798695 Neutrophil degranulation
R-HSA-9033241 Peroxisomal protein import
R-HSA-9033500 TYSND1 cleaves peroxisomal proteins
UniPathwayi
UPA00199

Names & Taxonomyi

Protein namesi
Recommended name:
3-ketoacyl-CoA thiolase, peroxisomal (EC:2.3.1.16)
Alternative name(s):
Acetyl-CoA acyltransferase
Beta-ketothiolase
Peroxisomal 3-oxoacyl-CoA thiolase
Gene namesi
Name:ACAA1
Synonyms:ACAA, PTHIO
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000060971.17
HGNCiHGNC:82 ACAA1
MIMi604054 gene
neXtProtiNX_P09110

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Peroxisome

Pathology & Biotechi

Organism-specific databases

DisGeNETi30
OpenTargetsiENSG00000060971
PharmGKBiPA24419

Chemistry databases

DrugBankiDB09069 Trimetazidine

Polymorphism and mutation databases

BioMutaiACAA1
DMDMi135751

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 26PeroxisomeCombined sourcesAdd BLAST26
ChainiPRO_000003406727 – 4243-ketoacyl-CoA thiolase, peroxisomalAdd BLAST398

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei59PhosphothreonineCombined sources1
Modified residuei60PhosphothreonineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP09110
MaxQBiP09110
PaxDbiP09110
PeptideAtlasiP09110
PRIDEiP09110
ProteomicsDBi52200

2D gel databases

REPRODUCTION-2DPAGEiIPI00012828
UCD-2DPAGEiP09110

PTM databases

CarbonylDBiP09110
iPTMnetiP09110
PhosphoSitePlusiP09110
SwissPalmiP09110

Expressioni

Inductioni

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.

Gene expression databases

BgeeiENSG00000060971 Expressed in 236 organ(s), highest expression level in nephron tubule
CleanExiHS_ACAA1
ExpressionAtlasiP09110 baseline and differential
GenevisibleiP09110 HS

Organism-specific databases

HPAiHPA006764
HPA007244

Interactioni

Subunit structurei

Homodimer.1 Publication

Protein-protein interaction databases

BioGridi106548, 32 interactors
IntActiP09110, 7 interactors
STRINGi9606.ENSP00000333664

Structurei

Secondary structure

1424
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP09110
SMRiP09110
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP09110

Family & Domainsi

Sequence similaritiesi

Belongs to the thiolase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1389 Eukaryota
COG0183 LUCA
GeneTreeiENSGT00930000151024
HOGENOMiHOG000012239
HOVERGENiHBG003112
InParanoidiP09110
KOiK07513
OMAiRYGLTTM
OrthoDBiEOG091G09C6
PhylomeDBiP09110
TreeFamiTF332308

Family and domain databases

CDDicd00751 thiolase, 1 hit
Gene3Di3.40.47.10, 1 hit
InterProiView protein in InterPro
IPR002155 Thiolase
IPR016039 Thiolase-like
IPR020615 Thiolase_acyl_enz_int_AS
IPR020610 Thiolase_AS
IPR020617 Thiolase_C
IPR020613 Thiolase_CS
IPR020616 Thiolase_N
PfamiView protein in Pfam
PF02803 Thiolase_C, 1 hit
PF00108 Thiolase_N, 1 hit
PIRSFiPIRSF000429 Ac-CoA_Ac_transf, 1 hit
SUPFAMiSSF53901 SSF53901, 2 hits
TIGRFAMsiTIGR01930 AcCoA-C-Actrans, 1 hit
PROSITEiView protein in PROSITE
PS00098 THIOLASE_1, 1 hit
PS00737 THIOLASE_2, 1 hit
PS00099 THIOLASE_3, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 6 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P09110-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MQRLQVVLGH LRGPADSGWM PQAAPCLSGA PQASAADVVV VHGRRTAICR
60 70 80 90 100
AGRGGFKDTT PDELLSAVMT AVLKDVNLRP EQLGDICVGN VLQPGAGAIM
110 120 130 140 150
ARIAQFLSDI PETVPLSTVN RQCSSGLQAV ASIAGGIRNG SYDIGMACGV
160 170 180 190 200
ESMSLADRGN PGNITSRLME KEKARDCLIP MGITSENVAE RFGISREKQD
210 220 230 240 250
TFALASQQKA ARAQSKGCFQ AEIVPVTTTV HDDKGTKRSI TVTQDEGIRP
260 270 280 290 300
STTMEGLAKL KPAFKKDGST TAGNSSQVSD GAAAILLARR SKAEELGLPI
310 320 330 340 350
LGVLRSYAVV GVPPDIMGIG PAYAIPVALQ KAGLTVSDVD IFEINEAFAS
360 370 380 390 400
QAAYCVEKLR LPPEKVNPLG GAVALGHPLG CTGARQVITL LNELKRRGKR
410 420
AYGVVSMCIG TGMGAAAVFE YPGN
Length:424
Mass (Da):44,292
Last modified:October 1, 1989 - v2
Checksum:i71B2BBAFA06AE412
GO
Isoform 2 (identifier: P09110-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     149-181: Missing.
     272-331: Missing.

Note: No experimental confirmation available.
Show »
Length:331
Mass (Da):34,665
Checksum:i8137A0AC448FBA24
GO

Computationally mapped potential isoform sequencesi

There are 6 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
C9JDE9C9JDE9_HUMAN
3-ketoacyl-CoA thiolase, peroxisoma...
ACAA1
383Annotation score:
H7C131H7C131_HUMAN
3-ketoacyl-CoA thiolase, peroxisoma...
ACAA1
290Annotation score:
H0Y4D4H0Y4D4_HUMAN
3-ketoacyl-CoA thiolase, peroxisoma...
ACAA1
237Annotation score:
B4DVF4B4DVF4_HUMAN
3-ketoacyl-CoA thiolase, peroxisoma...
ACAA1
153Annotation score:
F2Z2Q6F2Z2Q6_HUMAN
3-ketoacyl-CoA thiolase, peroxisoma...
ACAA1
113Annotation score:
F2Z3P7F2Z3P7_HUMAN
3-ketoacyl-CoA thiolase, peroxisoma...
ACAA1
121Annotation score:

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_011904172E → D. Corresponds to variant dbSNP:rs156265Ensembl.1
Natural variantiVAR_069148387V → A1 PublicationCorresponds to variant dbSNP:rs2229528Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_046195149 – 181Missing in isoform 2. 1 PublicationAdd BLAST33
Alternative sequenceiVSP_046196272 – 331Missing in isoform 2. 1 PublicationAdd BLAST60

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X12966 mRNA Translation: CAA31412.1
X14813 mRNA Translation: CAA32918.1
X65140 Genomic DNA Translation: CAA46270.1
X65148 Genomic DNA Translation: CAA46271.1
AP006309 Genomic DNA No translation available.
CH471055 Genomic DNA Translation: EAW64516.1
BC000635 mRNA Translation: AAH00635.1
BC011977 mRNA Translation: AAH11977.1
BC014474 mRNA Translation: AAH14474.1
CCDSiCCDS2673.1 [P09110-1]
CCDS46794.1 [P09110-2]
PIRiS17515 XUHUAB
RefSeqiNP_001123882.1, NM_001130410.1 [P09110-2]
NP_001598.1, NM_001607.3 [P09110-1]
UniGeneiHs.643487

Genome annotation databases

EnsembliENST00000301810; ENSP00000301810; ENSG00000060971 [P09110-2]
ENST00000333167; ENSP00000333664; ENSG00000060971 [P09110-1]
GeneIDi30
KEGGihsa:30
UCSCiuc003cht.4 human [P09110-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X12966 mRNA Translation: CAA31412.1
X14813 mRNA Translation: CAA32918.1
X65140 Genomic DNA Translation: CAA46270.1
X65148 Genomic DNA Translation: CAA46271.1
AP006309 Genomic DNA No translation available.
CH471055 Genomic DNA Translation: EAW64516.1
BC000635 mRNA Translation: AAH00635.1
BC011977 mRNA Translation: AAH11977.1
BC014474 mRNA Translation: AAH14474.1
CCDSiCCDS2673.1 [P09110-1]
CCDS46794.1 [P09110-2]
PIRiS17515 XUHUAB
RefSeqiNP_001123882.1, NM_001130410.1 [P09110-2]
NP_001598.1, NM_001607.3 [P09110-1]
UniGeneiHs.643487

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2IIKX-ray2.55A/B30-423[»]
ProteinModelPortaliP09110
SMRiP09110
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi106548, 32 interactors
IntActiP09110, 7 interactors
STRINGi9606.ENSP00000333664

Chemistry databases

DrugBankiDB09069 Trimetazidine

PTM databases

CarbonylDBiP09110
iPTMnetiP09110
PhosphoSitePlusiP09110
SwissPalmiP09110

Polymorphism and mutation databases

BioMutaiACAA1
DMDMi135751

2D gel databases

REPRODUCTION-2DPAGEiIPI00012828
UCD-2DPAGEiP09110

Proteomic databases

EPDiP09110
MaxQBiP09110
PaxDbiP09110
PeptideAtlasiP09110
PRIDEiP09110
ProteomicsDBi52200

Protocols and materials databases

DNASUi30
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000301810; ENSP00000301810; ENSG00000060971 [P09110-2]
ENST00000333167; ENSP00000333664; ENSG00000060971 [P09110-1]
GeneIDi30
KEGGihsa:30
UCSCiuc003cht.4 human [P09110-1]

Organism-specific databases

CTDi30
DisGeNETi30
EuPathDBiHostDB:ENSG00000060971.17
GeneCardsiACAA1
HGNCiHGNC:82 ACAA1
HPAiHPA006764
HPA007244
MIMi604054 gene
neXtProtiNX_P09110
OpenTargetsiENSG00000060971
PharmGKBiPA24419
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1389 Eukaryota
COG0183 LUCA
GeneTreeiENSGT00930000151024
HOGENOMiHOG000012239
HOVERGENiHBG003112
InParanoidiP09110
KOiK07513
OMAiRYGLTTM
OrthoDBiEOG091G09C6
PhylomeDBiP09110
TreeFamiTF332308

Enzyme and pathway databases

UniPathwayi
UPA00199

BioCyciMetaCyc:HS00752-MONOMER
ReactomeiR-HSA-2046106 alpha-linolenic acid (ALA) metabolism
R-HSA-390247 Beta-oxidation of very long chain fatty acids
R-HSA-6798695 Neutrophil degranulation
R-HSA-9033241 Peroxisomal protein import
R-HSA-9033500 TYSND1 cleaves peroxisomal proteins

Miscellaneous databases

ChiTaRSiACAA1 human
EvolutionaryTraceiP09110
GeneWikiiACAA1
GenomeRNAii30
PROiPR:P09110
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000060971 Expressed in 236 organ(s), highest expression level in nephron tubule
CleanExiHS_ACAA1
ExpressionAtlasiP09110 baseline and differential
GenevisibleiP09110 HS

Family and domain databases

CDDicd00751 thiolase, 1 hit
Gene3Di3.40.47.10, 1 hit
InterProiView protein in InterPro
IPR002155 Thiolase
IPR016039 Thiolase-like
IPR020615 Thiolase_acyl_enz_int_AS
IPR020610 Thiolase_AS
IPR020617 Thiolase_C
IPR020613 Thiolase_CS
IPR020616 Thiolase_N
PfamiView protein in Pfam
PF02803 Thiolase_C, 1 hit
PF00108 Thiolase_N, 1 hit
PIRSFiPIRSF000429 Ac-CoA_Ac_transf, 1 hit
SUPFAMiSSF53901 SSF53901, 2 hits
TIGRFAMsiTIGR01930 AcCoA-C-Actrans, 1 hit
PROSITEiView protein in PROSITE
PS00098 THIOLASE_1, 1 hit
PS00737 THIOLASE_2, 1 hit
PS00099 THIOLASE_3, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiTHIK_HUMAN
AccessioniPrimary (citable) accession number: P09110
Secondary accession number(s): G5E935, Q96CA6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: October 1, 1989
Last modified: November 7, 2018
This is version 201 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  7. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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