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Protein

Peroxisomal acyl-coenzyme A oxidase 3

Gene

ACOX3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Oxidizes the CoA-esters of 2-methyl-branched fatty acids.By similarity

Catalytic activityi

Acyl-CoA + O2 = trans-2,3-dehydroacyl-CoA + H2O2.

Cofactori

FADBy similarity

Pathway:iperoxisomal fatty acid beta-oxidation

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

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Fatty acid metabolism, Lipid metabolism

Keywords - Ligandi

FAD, Flavoprotein

Enzyme and pathway databases

ReactomeiREACT_17017. Beta-oxidation of pristanoyl-CoA.
UniPathwayiUPA00661.

Names & Taxonomyi

Protein namesi
Recommended name:
Peroxisomal acyl-coenzyme A oxidase 3 (EC:1.3.3.6)
Alternative name(s):
Branched-chain acyl-CoA oxidase
Short name:
BRCACox
Pristanoyl-CoA oxidase
Gene namesi
Name:ACOX3
Synonyms:BRCOX, PRCOX
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:121. ACOX3.

Subcellular locationi

GO - Cellular componenti

  • membrane Source: UniProtKB
  • mitochondrion Source: Ensembl
  • peroxisomal matrix Source: Reactome
  • peroxisome Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Peroxisome

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA24445.

Polymorphism and mutation databases

BioMutaiACOX3.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methioninei1 – 11Removed2 Publications
Chaini2 – 700699Peroxisomal acyl-coenzyme A oxidase 3PRO_0000204685Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylalanine2 Publications
Modified residuei281 – 2811Phosphothreonine1 Publication

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiO15254.
PaxDbiO15254.
PRIDEiO15254.

PTM databases

PhosphoSiteiO15254.

Expressioni

Gene expression databases

BgeeiO15254.
CleanExiHS_ACOX3.
ExpressionAtlasiO15254. baseline and differential.
GenevisibleiO15254. HS.

Organism-specific databases

HPAiHPA035840.
HPA035841.

Interactioni

Protein-protein interaction databases

BioGridi113907. 10 interactions.
IntActiO15254. 1 interaction.
MINTiMINT-1192218.
STRINGi9606.ENSP00000348775.

Structurei

3D structure databases

ProteinModelPortaliO15254.
SMRiO15254. Positions 123-673.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi698 – 7003Microbody targeting signalBy similarity

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi340 – 3434Poly-Glu

Sequence similaritiesi

Belongs to the acyl-CoA oxidase family.Curated

Phylogenomic databases

eggNOGiCOG1960.
GeneTreeiENSGT00530000062919.
HOGENOMiHOG000245077.
HOVERGENiHBG101107.
InParanoidiO15254.
KOiK00232.
OMAiCTHAIVY.
OrthoDBiEOG744T8F.
PhylomeDBiO15254.
TreeFamiTF314226.

Family and domain databases

Gene3Di2.40.110.10. 1 hit.
InterProiIPR006091. Acyl-CoA_Oxase/DH_cen-dom.
IPR012258. Acyl-CoA_oxidase.
IPR002655. Acyl-CoA_oxidase_C.
IPR009075. AcylCo_DH/oxidase_C.
IPR009100. AcylCoA_DH/oxidase_NM_dom.
[Graphical view]
PfamiPF01756. ACOX. 1 hit.
PF00441. Acyl-CoA_dh_1. 1 hit.
PF02770. Acyl-CoA_dh_M. 1 hit.
[Graphical view]
PIRSFiPIRSF000168. Acyl-CoA_oxidase. 1 hit.
SUPFAMiSSF47203. SSF47203. 2 hits.
SSF56645. SSF56645. 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

Isoform 1 (identifier: O15254-1) [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
MASTVEGGDT ALLPEFPRGP LDAYRARASF SWKELALFTE GEGMLRFKKT
60 70 80 90 100
IFSALENDPL FARSPGADLS LEKYRELNFL RCKRIFEYDF LSVEDMFKSP
110 120 130 140 150
LKVPALIQCL GMYDSSLAAK YLLHSLVFGS AVYSSGSERH LTYIQKIFRM
160 170 180 190 200
EIFGCFALTE LSHGSNTKAI RTTAHYDPAT EEFIIHSPDF EAAKFWVGNM
210 220 230 240 250
GKTATHAVVF AKLCVPGDQC HGLHPFIVQI RDPKTLLPMP GVMVGDIGKK
260 270 280 290 300
LGQNGLDNGF AMFHKVRVPR QSLLNRMGDV TPEGTYVSPF KDVRQRFGAS
310 320 330 340 350
LGSLSSGRVS IVSLAILNLK LAVAIALRFS ATRRQFGPTE EEEIPVLEYP
360 370 380 390 400
MQQWRLLPYL AAVYALDHFS KSLFLDLVEL QRGLASGDRS ARQAELGREI
410 420 430 440 450
HALASASKPL ASWTTQQGIQ ECREACGGHG YLAMNRLGVL RDDNDPNCTY
460 470 480 490 500
EGDNNILLQQ TSNYLLGLLA HQVHDGACFR SPLKSVDFLD AYPGILDQKF
510 520 530 540 550
EVSSVADCLD SAVALAAYKW LVCYLLRETY QKLNQEKRSG SSDFEARNKC
560 570 580 590 600
QVSHGRPLAL AFVELTVVQR FHEHVHQPSV PPSLRAVLGR LSALYALWSL
610 620 630 640 650
SRHAALLYRG GYFSGEQAGE VLESAVLALC SQLKDDAVAL VDVIAPPDFV
660 670 680 690 700
LDSPIGRADG ELYKNLWGAV LQESKVLERA SWWPEFSVNK PVIGSLKSKL
Length:700
Mass (Da):77,629
Last modified:February 20, 2007 - v2
Checksum:i40D1CD3DC6A620C1
GO
Isoform 2 (identifier: O15254-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     610-624: GGYFSGEQAGEVLES → AERRCSCPGRRDRSS
     625-700: Missing.

Note: No experimental confirmation available.
Show »
Length:624
Mass (Da):69,575
Checksum:i1894338CCBF1939A
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti34 – 352EL → DV in CAA72214 (PubMed:9271077).Curated
Sequence conflicti44 – 452ML → NV in CAA72214 (PubMed:9271077).Curated
Sequence conflicti95 – 951D → A in CAA72214 (PubMed:9271077).Curated
Sequence conflicti365 – 3651A → G in CAA72214 (PubMed:9271077).Curated
Sequence conflicti601 – 6011S → K in CAA72214 (PubMed:9271077).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti34 – 341E → A.
Corresponds to variant rs12513296 [ dbSNP | Ensembl ].
VAR_030802
Natural varianti497 – 4971D → N.
Corresponds to variant rs13434465 [ dbSNP | Ensembl ].
VAR_030803

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei610 – 62415GGYFS…EVLES → AERRCSCPGRRDRSS in isoform 2. 1 PublicationVSP_023355Add
BLAST
Alternative sequencei625 – 70076Missing in isoform 2. 1 PublicationVSP_023356Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y11411 mRNA. Translation: CAA72214.1.
BC017053 mRNA. Translation: AAH17053.1.
CCDSiCCDS3401.1. [O15254-1]
CCDS47017.1. [O15254-2]
RefSeqiNP_001095137.1. NM_001101667.1. [O15254-2]
NP_003492.2. NM_003501.2. [O15254-1]
XP_005248068.1. XM_005248011.3. [O15254-1]
XP_005248070.1. XM_005248013.3. [O15254-2]
XP_011511867.1. XM_011513565.1. [O15254-1]
UniGeneiHs.479122.

Genome annotation databases

EnsembliENST00000356406; ENSP00000348775; ENSG00000087008.
ENST00000413009; ENSP00000413994; ENSG00000087008. [O15254-2]
ENST00000503233; ENSP00000421625; ENSG00000087008.
GeneIDi8310.
KEGGihsa:8310.
UCSCiuc003glc.4. human. [O15254-1]
uc003gld.4. human. [O15254-2]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y11411 mRNA. Translation: CAA72214.1.
BC017053 mRNA. Translation: AAH17053.1.
CCDSiCCDS3401.1. [O15254-1]
CCDS47017.1. [O15254-2]
RefSeqiNP_001095137.1. NM_001101667.1. [O15254-2]
NP_003492.2. NM_003501.2. [O15254-1]
XP_005248068.1. XM_005248011.3. [O15254-1]
XP_005248070.1. XM_005248013.3. [O15254-2]
XP_011511867.1. XM_011513565.1. [O15254-1]
UniGeneiHs.479122.

3D structure databases

ProteinModelPortaliO15254.
SMRiO15254. Positions 123-673.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113907. 10 interactions.
IntActiO15254. 1 interaction.
MINTiMINT-1192218.
STRINGi9606.ENSP00000348775.

PTM databases

PhosphoSiteiO15254.

Polymorphism and mutation databases

BioMutaiACOX3.

Proteomic databases

MaxQBiO15254.
PaxDbiO15254.
PRIDEiO15254.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000356406; ENSP00000348775; ENSG00000087008.
ENST00000413009; ENSP00000413994; ENSG00000087008. [O15254-2]
ENST00000503233; ENSP00000421625; ENSG00000087008.
GeneIDi8310.
KEGGihsa:8310.
UCSCiuc003glc.4. human. [O15254-1]
uc003gld.4. human. [O15254-2]

Organism-specific databases

CTDi8310.
GeneCardsiGC04M008368.
HGNCiHGNC:121. ACOX3.
HPAiHPA035840.
HPA035841.
MIMi603402. gene.
neXtProtiNX_O15254.
PharmGKBiPA24445.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG1960.
GeneTreeiENSGT00530000062919.
HOGENOMiHOG000245077.
HOVERGENiHBG101107.
InParanoidiO15254.
KOiK00232.
OMAiCTHAIVY.
OrthoDBiEOG744T8F.
PhylomeDBiO15254.
TreeFamiTF314226.

Enzyme and pathway databases

UniPathwayiUPA00661.
ReactomeiREACT_17017. Beta-oxidation of pristanoyl-CoA.

Miscellaneous databases

ChiTaRSiACOX3. human.
GeneWikiiACOX3.
GenomeRNAii8310.
NextBioi31121.
PROiO15254.
SOURCEiSearch...

Gene expression databases

BgeeiO15254.
CleanExiHS_ACOX3.
ExpressionAtlasiO15254. baseline and differential.
GenevisibleiO15254. HS.

Family and domain databases

Gene3Di2.40.110.10. 1 hit.
InterProiIPR006091. Acyl-CoA_Oxase/DH_cen-dom.
IPR012258. Acyl-CoA_oxidase.
IPR002655. Acyl-CoA_oxidase_C.
IPR009075. AcylCo_DH/oxidase_C.
IPR009100. AcylCoA_DH/oxidase_NM_dom.
[Graphical view]
PfamiPF01756. ACOX. 1 hit.
PF00441. Acyl-CoA_dh_1. 1 hit.
PF02770. Acyl-CoA_dh_M. 1 hit.
[Graphical view]
PIRSFiPIRSF000168. Acyl-CoA_oxidase. 1 hit.
SUPFAMiSSF47203. SSF47203. 2 hits.
SSF56645. SSF56645. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Evidence for the existence of a pristanoyl-CoA oxidase gene in man."
    Vanhooren J.C.T., Marynen P., Mannaerts G.P., van Veldhoven P.P.
    Biochem. J. 325:593-599(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), FUNCTION.
    Tissue: Liver.
  2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
    Tissue: Uterus.
  3. Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT THR-281, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  4. "Comparative large-scale characterisation of plant vs. mammal proteins reveals similar and idiosyncratic N-alpha acetylation features."
    Bienvenut W.V., Sumpton D., Martinez A., Lilla S., Espagne C., Meinnel T., Giglione C.
    Mol. Cell. Proteomics 11:M111.015131-M111.015131(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT ALA-2, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS], CLEAVAGE OF INITIATOR METHIONINE [LARGE SCALE ANALYSIS].
  5. Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT ALA-2, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS], CLEAVAGE OF INITIATOR METHIONINE [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiACOX3_HUMAN
AccessioniPrimary (citable) accession number: O15254
Secondary accession number(s): Q96AJ8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 2, 2001
Last sequence update: February 20, 2007
Last modified: July 22, 2015
This is version 132 of the entry and version 2 of the sequence. [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

Complete proteome, Reference proteome

Documents

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

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.