Rabbit anti-Human ACOX2 Polyclonal Antibody

Uniprot Id

Q99424

Target Species

Human

Target Name

ACOX2

Target Full Name

Peroxisomal acyl-coenzyme A oxidase 2

Target Function

Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycholestanoic acids. Capable of oxidizing short as well as long chain 2-methyl branched fatty acids.

Target Involvement

Congenital bile acid synthesis defect 6 (CBAS6)

Target Subcellular Location

Peroxisome.

Target Protein Families

Acyl-CoA oxidase family

Target Tissue Specificity

Present in all tissues tested: heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Most abundant in heart, liver and kidney.

Target Synonyms

12-alpha-trihydroxy-5-beta-cholestanoyl-CoA 24-hydroxylase; 12-alpha-trihydroxy-5-beta-cholestanoyl-CoA oxidase; 3 alpha 7 alpha 12 alpha trihydroxy 5 beta cholestanoyl CoA oxidase; 3 alpha 7 alpha12 alpha trihydroxy 5 beta cholestanoyl CoA 24 hydroxylase; 3-alpha; 7-alpha; ACOX 2; ACOX2; ACOX2_HUMAN; Acyl CoA oxidase 2; Acyl Coenzyme A oxidase 2; Acyl Coenzyme A oxidase 2 branched chain; Acyl coenzyme A oxidase 2 peroxisomal; BCOX; BRCACOX; BRCOX; Peroxisomal acyl-coenzyme A oxidase 2; Peroxisomal branched chain acyl CoA oxidase; THCA CoA oxidase; THCA-CoA oxidase; THCCox; Trihydroxycoprostanoyl CoA oxidase; Trihydroxycoprostanoyl-CoA oxidase

Target Background

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.