The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified.It has 3 isoforms produced by alternative splicing with the molecular mass of 70-74 kDa and can be cleaved post-translationally into a 50-kDa and a 22-kDa subunit between Val468 and Ala469 (PMID: 10672038).
Categories
Primary Antibodies
Cellular Localization
Peroxisome
Clonality
polyclonal
Description
Catalyzes the desaturation of acyl-CoAs to 2-trans- enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy- palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl- CoA.
Host
Rabbit
Immunogen
acyl-Coenzyme A oxidase 1, palmitoyl
Isotype
IgG
Molecular Weight
74 kDa
Reactivity
Human, Mouse, Rat
Regulatory
RUO
Subcellular Location
Peroxisome
Synonyms
ACOX, ACOX1, AOX, PALMCOX, Palmitoyl CoA oxidase, SCOX
Uniprot
Q15067
Gene Id
51
Research Area
Developmental biology, Metabolism
Weight
74kDa,observed 50kDa
Form
liquid
Format
liquid
Purification
Immunogen affinity purified
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)