ACOX2


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 cognitive disability, and death in children. [provided by RefSeq, Mar 2009].

NameOMIM IDEnsembl IDHGNC IDPHARMGKB IDMap Location
acyl-CoA oxidase 2MIM:601641Ensembl:ENSG00000168306HGNC:HGNC:120PA244443p14.3

GO terms in ACOX2


Term TypeEvidence TypeGO Term IDGO Des.
MFIBAGO:0003995acyl-CoA dehydrogenase activity
MFIBAGO:0003997acyl-CoA oxidase activity
MFTASGO:0003997acyl-CoA oxidase activity
MFIPIGO:0005102signaling receptor binding
MFIBAGO:0005504fatty acid binding
MFTASGO:0016402pristanoyl-CoA oxidase activity
MFIBAGO:00337913alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase activity
MFIDAGO:00337913alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase activity
MFISSGO:00337913alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase activity
MFISSGO:0042803protein homodimerization activity
MFIBAGO:0050660flavin adenine dinucleotide binding
MFISSGO:0050660flavin adenine dinucleotide binding
MFIEAGO:0071949FAD binding
CCIBAGO:0005777peroxisome
CCIDAGO:0005777peroxisome
CCIMPGO:0005777peroxisome
CCISSGO:0005777peroxisome
CCTASGO:0005782peroxisomal matrix
CCIDAGO:0005829cytosol
CCTASGO:0005829cytosol
CCIDAGO:0043231intracellular membrane-bounded organelle
BPTASGO:0006625protein targeting to peroxisome
BPTASGO:0006699bile acid biosynthetic process
BPIDAGO:0010942positive regulation of cell death
BPIBAGO:0033539fatty acid beta-oxidation using acyl-CoA dehydrogenase
BPIBAGO:0033540fatty acid beta-oxidation using acyl-CoA oxidase
BPIDAGO:0033540fatty acid beta-oxidation using acyl-CoA oxidase
BPIMPGO:0033540fatty acid beta-oxidation using acyl-CoA oxidase
BPTASGO:0033540fatty acid beta-oxidation using acyl-CoA oxidase
BPIBAGO:0055088lipid homeostasis
BPIDAGO:1902884positive regulation of response to oxidative stress

Gene expression in normal tissue: ACOX2

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in ACOX2


DatabasePathway IDPathway Des.
wikipathwaysWP2435Quercetin and Nf-kB- AP-1 Induced Cell Apoptosis
wikipathwaysWP3942PPAR signaling pathway
smpdbSMP00035Bile Acid Biosynthesis
smpdbSMP00314Congenital Bile Acid Synthesis Defect Type II
smpdbSMP00315Cerebrotendinous Xanthomatosis (CTX)
smpdbSMP00316Zellweger Syndrome
smpdbSMP00317Familial Hypercholanemia (FHCA)
smpdbSMP00318Congenital Bile Acid Synthesis Defect Type III
smpdbSMP0072027-Hydroxylase Deficiency
kegghsa00120Primary bile acid biosynthesis - Homo sapiens (human)
kegghsa03320PPAR signaling pathway - Homo sapiens (human)
kegghsa04146Peroxisome - Homo sapiens (human)
humancycPWY-6061bile acid biosynthesis, neutral pathway
humancycPWY66-391fatty acid β-oxidation (peroxisome)
reactomeR-HSA-1430728Metabolism
reactomeR-HSA-192105Synthesis of bile acids and bile salts
reactomeR-HSA-193368Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol
reactomeR-HSA-194068Bile acid and bile salt metabolism
reactomeR-HSA-389887Beta-oxidation of pristanoyl-CoA
reactomeR-HSA-390918Peroxisomal lipid metabolism
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-556833Metabolism of lipids
reactomeR-HSA-8957322Metabolism of steroids
reactomeR-HSA-8978868Fatty acid metabolism
reactomeR-HSA-9033241Peroxisomal protein import
reactomeR-HSA-9033241Peroxisomal protein import

Gene-Drug: Aster Plot


Drug IDDrug NameModel Num.
iGMDRD30917AAG1
iGMDRD353PD03259011

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in ACOX2

​​​​