Gene/Proteome Database (LMPD)

LMPD ID
LMP000966
Gene ID
8309
Species
Homo sapiens (Human)
Gene Name
acyl-CoA oxidase 2, branched chain
Gene Symbol
ACOX2
Synonyms
BCOX; BRCACOX; BRCOX; THCCox
Alternate Names
peroxisomal acyl-coenzyme A oxidase 2; THCA-CoA oxidase; trihydroxycoprostanoyl-CoA oxidase; acyl-Coenzyme A oxidase 2, branched chain; peroxisomal branched chain acyl-CoA oxidase; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA oxidase; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA 24-hydroxylase
Chromosome
3
Map Location
3p14.3
EC Number
1.17.99.3
Summary
The product of this gene belongs to the acyl-CoA oxidase family. It encodes the branched-chain acyl-CoA oxidase which is involved in the degradation of long branched fatty acids and bile acid intermediates in peroxisomes. Deficiency of this enzyme results in the accumulation of branched fatty acids and bile acid intermediates, and may lead to Zellweger syndrome, severe mental retardation, and death in children. [provided by RefSeq, Mar 2009]
Orthologs
View orthologs and multiple alignments for ACOX2

Proteins

peroxisomal acyl-coenzyme A oxidase 2
Refseq ID NP_003491
Protein GI 4501869
UniProt ID Q99424
mRNA ID NM_003500
Length 681
RefSeq Status REVIEWED
MGSPVHRVSLGDTWSRQMHPDIESERYMQSFDVERLTNILDGGAQNTALRRKVESIIHSYPEFSCKDNYFMTQNERYKAAMRRAFHIRLIARRLGWLEDGRELGYAYRALSGDVALNIHRVFVRALRSLGSEEQIAKWDPLCKNIQIIATYAQTELGHGTYLQGLETEATYDAATQEFVIHSPTLTATKWWPGDLGRSATHALVQAQLICSGARRGMHAFIVPIRSLQDHTPLPGIIIGDIGPKMDFDQTDNGFLQLNHVRVPRENMLSRFAQVLPDGTYVKLGTAQSNYLPMVVVRVELLSGEILPILQKACVIAMRYSVIRRQSRLRPSDPEAKVLDYQTQQQKLFPQLAISYAFHFLAVSLLEFFQHSYTAILNQDFSFLPELHALSTGMKAMMSEFCTQGAEMCRRACGGHGYSKLSGLPSLVTKLSASCTYEGENTVLYLQVARFLVKSYLQTQMSPGSTPQRSLSPSVAYLTAPDLARCPAQRAADFLCPELYTTAWAHVAVRLIKDSVQHLQTLTQSGADQHEAWNQTTVIHLQAAKVHCYYVTVKGFTEALEKLENEPAIQQVLKRLCDLHAIHGILTNSGDFLHDAFLSGAQVDMARTAYLDLLRLIRKDAILLTDAFDFTDQCLNSALGCYDGNVYERLFQWAQKSPTNTQENPAYEEYIRPLLQSWRSKL

Gene Information

Entrez Gene ID
8309
Gene Name
acyl-CoA oxidase 2, branched chain
Gene Symbol
ACOX2
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005782 TAS:Reactome C peroxisomal matrix
GO:0005777 IDA:UniProtKB C peroxisome
GO:0033791 IEA:UniProtKB-EC F 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase activity
GO:0003995 IEA:InterPro F acyl-CoA dehydrogenase activity
GO:0003997 TAS:Reactome F acyl-CoA oxidase activity
GO:0050660 IEA:InterPro F flavin adenine dinucleotide binding
GO:0016402 TAS:Reactome F pristanoyl-CoA oxidase activity
GO:0005102 IPI:UniProtKB F receptor binding
GO:0006699 TAS:Reactome P bile acid biosynthetic process
GO:0008206 TAS:Reactome P bile acid metabolic process
GO:0044255 TAS:Reactome P cellular lipid metabolic process
GO:0033540 TAS:Reactome P fatty acid beta-oxidation using acyl-CoA oxidase
GO:0044281 TAS:Reactome P small molecule metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa03320 PPAR signaling pathway
hsa04146 Peroxisome
hsa00120 Primary bile acid biosynthesis

BIOCYC Pathway Links

BIOCYC Pathway ID Description
PWY-6061 bile acid biosynthesis, neutral pathway

Domain Information

InterPro Annotations

Accession Description
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminal
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminal
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain
IPR012258 Acyl-CoA oxidase
IPR002655 Acyl-CoA oxidase, C-terminal
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domain
IPR029320 Acyl-coenzyme A oxidase, N-terminal

UniProt Annotations

Entry Information

Gene Name
acyl-CoA oxidase 2, branched chain
Protein Entry
ACOX2_HUMAN
UniProt ID
Q99424
Species
Human

Comments

Comment Type Description
Catalytic Activity (25R)-3-alpha,7-alpha,12-alpha-trihydroxy-5- beta-cholestan-26-oyl-CoA + H(2)O + acceptor = (24R,25R)-3- alpha,7-alpha,12-alpha,24-tetrahydroxy-5-beta-cholestan-26-oyl-CoA + reduced acceptor.
Cofactor Name=FAD; Xref=ChEBI
Function Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycholestanoic acids.
Similarity Belongs to the acyl-CoA oxidase family.
Subcellular Location Peroxisome.
Subunit Heterodimer.
Tissue Specificity Present in all tissues tested: heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Most abundant in heart, liver and kidney.

Identical and Related Proteins

Unique RefSeq proteins for LMP000966 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4501869 RefSeq NP_003491 681 peroxisomal acyl-coenzyme A oxidase 2

Identical Sequences to LMP000966 proteins

Reference Database Accession Length Protein Name
GI:4501869 EMBL CBU96030.1 681 unnamed protein product [Homo sapiens]
GI:4501869 GenBank AGX52707.1 681 Sequence 5769 from patent US 8541208
GI:4501869 GenBank AGX57644.1 681 Sequence 15631 from patent US 8541208
GI:4501869 GenBank AGX63428.1 681 Sequence 26960 from patent US 8541208
GI:4501869 GenBank AIC50078.1 681 ACOX2, partial [synthetic construct]
GI:4501869 RefSeq XP_005265562.1 681 PREDICTED: peroxisomal acyl-coenzyme A oxidase 2 isoform X1 [Homo sapiens]

Related Sequences to LMP000966 proteins

Reference Database Accession Length Protein Name
GI:4501869 GenBank EAW65377.1 683 acyl-Coenzyme A oxidase 2, branched chain, isoform CRA_c [Homo sapiens]
GI:4501869 RefSeq XP_516559.2 681 PREDICTED: peroxisomal acyl-coenzyme A oxidase 2 isoform X1 [Pan troglodytes]
GI:4501869 RefSeq XP_003811663.1 681 PREDICTED: peroxisomal acyl-coenzyme A oxidase 2 isoform X1 [Pan paniscus]
GI:4501869 RefSeq XP_004034432.1 681 PREDICTED: peroxisomal acyl-coenzyme A oxidase 2 [Gorilla gorilla gorilla]
GI:4501869 RefSeq XP_009237124.1 681 PREDICTED: peroxisomal acyl-coenzyme A oxidase 2 isoform X1 [Pongo abelii]
GI:4501869 RefSeq XP_009444005.1 681 PREDICTED: peroxisomal acyl-coenzyme A oxidase 2 isoform X1 [Pan troglodytes]