MGP Database

MGP003480

Record overview

MGPD IDMGP003480
Gene ID8309
SpeciesHomo sapiens (Human)
Gene Nameacyl-CoA oxidase 2, branched chain
Gene Symbol ACOX2
SynonymsBCOX; BRCOX; THCCox; BRCACOX;
Alternate namesperoxisomal acyl-coenzyme A oxidase 2; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA 24-hydroxylase; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA oxidase; THCA-CoA oxidase; acyl-Coenzyme A oxidase 2, branched chain; peroxisomal branched chain acyl-CoA oxidase; trihydroxycoprostanoyl-CoA oxidase;
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:
MGSPVHRVSLGDTWSRQMHPDIESERYMQSFDVERLTNILDGGAQNTALRRKVESIIHSYPEFSCKDNYFMTQNERYKAAMRRAFHIRLIARRLGWLEDG
RELGYAYRALSGDVALNIHRVFVRALRSLGSEEQIAKWDPLCKNIQIIATYAQTELGHGTYLQGLETEATYDAATQEFVIHSPTLTATKWWPGDLGRSAT
HALVQAQLICSGARRGMHAFIVPIRSLQDHTPLPGIIIGDIGPKMDFDQTDNGFLQLNHVRVPRENMLSRFAQVLPDGTYVKLGTAQSNYLPMVVVRVEL
LSGEILPILQKACVIAMRYSVIRRQSRLRPSDPEAKVLDYQTQQQKLFPQLAISYAFHFLAVSLLEFFQHSYTAILNQDFSFLPELHALSTGMKAMMSEF
CTQGAEMCRRACGGHGYSKLSGLPSLVTKLSASCTYEGENTVLYLQVARFLVKSYLQTQMSPGSTPQRSLSPSVAYLTAPDLARCPAQRAADFLCPELYT
TAWAHVAVRLIKDSVQHLQTLTQSGADQHEAWNQTTVIHLQAAKVHCYYVTVKGFTEALEKLENEPAIQQVLKRLCDLHAIHGILTNSGDFLHDAFLSGA
QVDMARTAYLDLLRLIRKDAILLTDAFDFTDQCLNSALGCYDGNVYERLFQWAQKSPTNTQENPAYEEYIRPLLQSWRSKL
 
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