LMPD Database

LMP000966

Record overview

LMPD IDLMP000966
Gene ID8309
SpeciesHomo sapiens (Human)
Gene Nameacyl-CoA oxidase 2, branched chain
Gene SymbolACOX2
SynonymsBCOX; BRCACOX; BRCOX; THCCox
Alternate namesperoxisomal 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
Chromosome3
Map Location3p14.3
EC Number1.17.99.3
SummaryThe 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]
OrthologsView 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
MGSPVHRVSLGDTWSRQMHPDIESERYMQSFDVERLTNILDGGAQNTALRRKVESIIHSYPEFSCKDNYFMTQNERYKAAMRRAFHIRLIARRLGWLEDG
RELGYAYRALSGDVALNIHRVFVRALRSLGSEEQIAKWDPLCKNIQIIATYAQTELGHGTYLQGLETEATYDAATQEFVIHSPTLTATKWWPGDLGRSAT
HALVQAQLICSGARRGMHAFIVPIRSLQDHTPLPGIIIGDIGPKMDFDQTDNGFLQLNHVRVPRENMLSRFAQVLPDGTYVKLGTAQSNYLPMVVVRVEL
LSGEILPILQKACVIAMRYSVIRRQSRLRPSDPEAKVLDYQTQQQKLFPQLAISYAFHFLAVSLLEFFQHSYTAILNQDFSFLPELHALSTGMKAMMSEF
CTQGAEMCRRACGGHGYSKLSGLPSLVTKLSASCTYEGENTVLYLQVARFLVKSYLQTQMSPGSTPQRSLSPSVAYLTAPDLARCPAQRAADFLCPELYT
TAWAHVAVRLIKDSVQHLQTLTQSGADQHEAWNQTTVIHLQAAKVHCYYVTVKGFTEALEKLENEPAIQQVLKRLCDLHAIHGILTNSGDFLHDAFLSGA
QVDMARTAYLDLLRLIRKDAILLTDAFDFTDQCLNSALGCYDGNVYERLFQWAQKSPTNTQENPAYEEYIRPLLQSWRSKL