NP_003491.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
76,827 Da
NCBI Official Full Name
peroxisomal acyl-coenzyme A oxidase 2
NCBI Official Synonym Full Names
acyl-CoA oxidase 2, branched chain
NCBI Protein Information
peroxisomal acyl-coenzyme A oxidase 2
UniProt Protein Name
Peroxisomal acyl-coenzyme A oxidase 2
UniProt Synonym Protein Names
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; Trihydroxycoprostanoyl-CoA oxidase; THCA-CoA oxidase; THCCox
UniProt Synonym Gene Names
UniProt Entry Name
ACOX2_HUMAN
NCBI Summary for ACOX2
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]
UniProt Comments for ACOX2
ACOX2: Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycholestanoic acids. Belongs to the acyl-CoA oxidase family.
Protein type: EC 1.17.99.3; Lipid Metabolism - primary bile acid biosynthesis; Oxidoreductase
Chromosomal Location of Human Ortholog: 3p14.3
Cellular Component: intracellular membrane-bound organelle; mitochondrion; peroxisomal matrix; peroxisome
Molecular Function: 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase activity; acyl-CoA binding; acyl-CoA dehydrogenase activity; acyl-CoA oxidase activity; electron carrier activity; FAD binding; fatty acid binding; pristanoyl-CoA oxidase activity; receptor binding
Biological Process: bile acid biosynthetic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; fatty acid beta-oxidation using acyl-CoA oxidase; lipid homeostasis
Research Articles on ACOX2
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Pathways associated with anti-ACOX2 antibody
Diseases associated with anti-ACOX2 antibody
Organs/Tissues associated with anti-ACOX2 antibody
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