O15254 (ACOX3_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

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 names

Name:	ACOX3
Synonyms:	BRCOX, PRCOX

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	700 AA.
Sequence status	Complete.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Oxidizes the CoA-esters of 2-methyl-branched fatty acids By similarity. Ref.1
Catalytic activity	Acyl-CoA + O2 = trans-2,3-dehydroacyl-CoA + H2O2.
Cofactor	FAD By similarity.
Pathway	Lipid metabolism; peroxisomal fatty acid beta-oxidation.
Subcellular location	Peroxisome Potential.
Sequence similarities	Belongs to the acyl-CoA oxidase family.

Ontologies

Keywords
Biological process	Fatty acid metabolism Lipid metabolism
Cellular component	Peroxisome
Coding sequence diversity	Alternative splicing Polymorphism
Ligand	FAD Flavoprotein
Molecular function	Oxidoreductase
PTM	Phosphoprotein
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	fatty acid beta-oxidation using acyl-CoA oxidase Traceable author statement. Source: Reactome
Cellular_component	mitochondrion Inferred from electronic annotation. Source: Compara peroxisomal matrix Traceable author statement. Source: Reactome
Molecular_function	acyl-CoA dehydrogenase activity Inferred from electronic annotation. Source: InterPro flavin adenine dinucleotide binding Inferred from electronic annotation. Source: InterPro pristanoyl-CoA oxidase activity Traceable author statement. Source: Reactome
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: O15254-1)

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.

Isoform 2 (identifier: O15254-2)

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

Note: No experimental confirmation available.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 700	700	Peroxisomal acyl-coenzyme A oxidase 3		PRO_0000204685
Regions
	Motif	698 – 700	3	Microbody targeting signal By similarity
	Compositional bias	340 – 343	4	Poly-Glu
Amino acid modifications
	Modified residue	281	1	Phosphothreonine Ref.3
Natural variations
	Alternative sequence	610 – 624	15	GGYFS…EVLES → AERRCSCPGRRDRSS in isoform 2.		VSP_023355
	Alternative sequence	625 – 700	76	Missing in isoform 2.		VSP_023356
	Natural variant	34	1	E → A. Corresponds to variant rs12513296 [ dbSNP | Ensembl ].		VAR_030802
	Natural variant	497	1	D → N. Corresponds to variant rs13434465 [ dbSNP | Ensembl ].		VAR_030803
Experimental info
	Sequence conflict	34 – 35	2	EL → DV in CAA72214. Ref.1
	Sequence conflict	44 – 45	2	ML → NV in CAA72214. Ref.1
	Sequence conflict	95	1	D → A in CAA72214. Ref.1
	Sequence conflict	365	1	A → G in CAA72214. Ref.1
	Sequence conflict	601	1	S → K in CAA72214. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified February 20, 2007. Version 2. Checksum: 40D1CD3DC6A620C1	FASTA	700	77,629
	10 20 30 40 50 60 MASTVEGGDT ALLPEFPRGP LDAYRARASF SWKELALFTE GEGMLRFKKT IFSALENDPL 70 80 90 100 110 120 FARSPGADLS LEKYRELNFL RCKRIFEYDF LSVEDMFKSP LKVPALIQCL GMYDSSLAAK 130 140 150 160 170 180 YLLHSLVFGS AVYSSGSERH LTYIQKIFRM EIFGCFALTE LSHGSNTKAI RTTAHYDPAT 190 200 210 220 230 240 EEFIIHSPDF EAAKFWVGNM GKTATHAVVF AKLCVPGDQC HGLHPFIVQI RDPKTLLPMP 250 260 270 280 290 300 GVMVGDIGKK LGQNGLDNGF AMFHKVRVPR QSLLNRMGDV TPEGTYVSPF KDVRQRFGAS 310 320 330 340 350 360 LGSLSSGRVS IVSLAILNLK LAVAIALRFS ATRRQFGPTE EEEIPVLEYP MQQWRLLPYL 370 380 390 400 410 420 AAVYALDHFS KSLFLDLVEL QRGLASGDRS ARQAELGREI HALASASKPL ASWTTQQGIQ 430 440 450 460 470 480 ECREACGGHG YLAMNRLGVL RDDNDPNCTY EGDNNILLQQ TSNYLLGLLA HQVHDGACFR 490 500 510 520 530 540 SPLKSVDFLD AYPGILDQKF EVSSVADCLD SAVALAAYKW LVCYLLRETY QKLNQEKRSG 550 560 570 580 590 600 SSDFEARNKC QVSHGRPLAL AFVELTVVQR FHEHVHQPSV PPSLRAVLGR LSALYALWSL 610 620 630 640 650 660 SRHAALLYRG GYFSGEQAGE VLESAVLALC SQLKDDAVAL VDVIAPPDFV LDSPIGRADG 670 680 690 700 ELYKNLWGAV LQESKVLERA SWWPEFSVNK PVIGSLKSKL
	Isoform 2 [UniParc]. Checksum: 1894338CCBF1939A	FASTA	624	69,575
	10 20 30 40 50 60 MASTVEGGDT ALLPEFPRGP LDAYRARASF SWKELALFTE GEGMLRFKKT IFSALENDPL 70 80 90 100 110 120 FARSPGADLS LEKYRELNFL RCKRIFEYDF LSVEDMFKSP LKVPALIQCL GMYDSSLAAK 130 140 150 160 170 180 YLLHSLVFGS AVYSSGSERH LTYIQKIFRM EIFGCFALTE LSHGSNTKAI RTTAHYDPAT 190 200 210 220 230 240 EEFIIHSPDF EAAKFWVGNM GKTATHAVVF AKLCVPGDQC HGLHPFIVQI RDPKTLLPMP 250 260 270 280 290 300 GVMVGDIGKK LGQNGLDNGF AMFHKVRVPR QSLLNRMGDV TPEGTYVSPF KDVRQRFGAS 310 320 330 340 350 360 LGSLSSGRVS IVSLAILNLK LAVAIALRFS ATRRQFGPTE EEEIPVLEYP MQQWRLLPYL 370 380 390 400 410 420 AAVYALDHFS KSLFLDLVEL QRGLASGDRS ARQAELGREI HALASASKPL ASWTTQQGIQ 430 440 450 460 470 480 ECREACGGHG YLAMNRLGVL RDDNDPNCTY EGDNNILLQQ TSNYLLGLLA HQVHDGACFR 490 500 510 520 530 540 SPLKSVDFLD AYPGILDQKF EVSSVADCLD SAVALAAYKW LVCYLLRETY QKLNQEKRSG 550 560 570 580 590 600 SSDFEARNKC QVSHGRPLAL AFVELTVVQR FHEHVHQPSV PPSLRAVLGR LSALYALWSL 610 620 SRHAALLYRA ERRCSCPGRR DRSS

References

	« Hide 'large scale' referencesShow 'large scale' references »
[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]	"Large-scale proteomics analysis of the human kinome." Oppermann F.S., Gnad F., Olsen J.V., Hornberger R., Greff Z., Keri G., Mann M., Daub H. Mol. Cell. Proteomics 8:1751-1764(2009) [PubMed] [Europe PMC] [Abstract] Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT THR-281, MASS SPECTROMETRY.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	Y11411 mRNA. Translation: CAA72214.1. BC017053 mRNA. Translation: AAH17053.1.
IPI	IPI00020226. IPI00827610.
RefSeq	NP_001095137.1. NM_001101667.1. NP_003492.2. NM_003501.2.
UniGene	Hs.479122.
3D structure databases
ProteinModelPortal	O15254.
ModBase	Search...
Protein-protein interaction databases
MINT	MINT-1192218.
STRING	9606.ENSP00000348775.
PTM databases
PhosphoSite	O15254.
Proteomic databases
PaxDb	O15254.
PRIDE	O15254.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000356406; ENSP00000348775; ENSG00000087008. ENST00000413009; ENSP00000413994; ENSG00000087008. ENST00000503233; ENSP00000421625; ENSG00000087008.
GeneID	8310.
KEGG	hsa:8310.
UCSC	uc003glc.4. human. uc003gld.4. human.
Organism-specific databases
CTD	8310.
GeneCards	GC04M008368.
HGNC	HGNC:121. ACOX3.
HPA	HPA035840. HPA035841.
MIM	603402. gene.
neXtProt	NX_O15254.
PharmGKB	PA24445.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG1960.
HOGENOM	HOG000245077.
HOVERGEN	HBG101107.
InParanoid	O15254.
KO	K00232.
OMA	PMQQWRL.
PhylomeDB	O15254.
Enzyme and pathway databases
Reactome	REACT_111217. Metabolism.
UniPathway	UPA00661.
Gene expression databases
ArrayExpress	O15254.
Bgee	O15254.
CleanEx	HS_ACOX3.
Genevestigator	O15254.
GermOnline	ENSG00000087008. Homo sapiens.
Family and domain databases
Gene3D	2.40.110.10. 1 hit.
InterPro	IPR006090. Acyl-CoA_Oxase/DH_1. IPR006091. Acyl-CoA_Oxase/DH_cen-dom. IPR012258. Acyl-CoA_oxidase. IPR002655. Acyl-CoA_oxidase_C. IPR009075. AcylCo_DH/oxidase_C. IPR009100. AcylCoA_DH/oxidase. [Graphical view]
PANTHER	PTHR10909:SF11. PTHR10909:SF11. 1 hit.
Pfam	PF01756. ACOX. 1 hit. PF00441. Acyl-CoA_dh_1. 1 hit. PF02770. Acyl-CoA_dh_M. 1 hit. [Graphical view]
PIRSF	PIRSF000168. Acyl-CoA_oxidase. 1 hit.
SUPFAM	SSF56645. AcylCoA_dehyd_NM. 1 hit. SSF47203. AcylCoADH_C_like. 2 hits.
ProtoNet	Search...
Other
ChiTaRS	ACOX3. human.
GenomeRNAi	8310.
NextBio	31121.
SOURCE	Search...

Entry information

Entry name

ACOX3_HUMAN

Accession

Primary (citable) accession number: O15254
Secondary accession number(s): Q96AJ8

Entry history

Integrated into UniProtKB/Swiss-Prot:	November 2, 2001
Last sequence update:	February 20, 2007
Last modified:	April 3, 2013
This is version 112 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.