catalog number :
MBS9403251
products full name :
ACOX1 Antibody
products short name :
[ACOX1]
products name syn :
[ACOX; SCOX; PALMCOX]
other names :
[peroxisomal acyl-coenzyme A oxidase 1 isoform c; Peroxisomal acyl-coenzyme A oxidase 1; peroxisomal acyl-coenzyme A oxidase 1; acyl-CoA oxidase 1, palmitoyl; Palmitoyl-CoA oxidase; Straight-chain acyl-CoA oxidase; SCOX]
products gene name :
[ACOX1]
other gene names :
[ACOX1; ACOX1; ACOX; SCOX; PALMCOX; ACOX; AOX; SCOX]
uniprot entry name :
ACOX1_HUMAN
reactivity :
Human, Mouse
specificity :
The antibody detects endogenous levels of total ACOX1 protein.
purity :
Antigen affinity purification.
form :
Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
concentration :
0.8 mg/ml
storage stability :
Store at -20 degree C
tested application :
Western Blot (WB), Immunohistochemistry (IHC)
app notes :
Western blotting: 1:200-1:1000. Immunohistochemistry: 1:15-1:50
image1 heading :
Testing Data
image2 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen Type: Recombinant Protein. Immunogen Description: Fusion protein corresponding to residues near the C terminal of human Acyl-CoA oxidase 1, palmitoyl
other info2 :
Target Name: ACOX1
products categories :
Total protein Ab
products description :
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.
ncbi acc num :
NP_001171968.1
ncbi gb acc num :
NM_001185039.1
ncbi pathways :
Beta-oxidation Of Very Long Chain Fatty Acids Pathway (1270034); Biosynthesis Of Unsaturated Fatty Acids Pathway (83034); Biosynthesis Of Unsaturated Fatty Acids Pathway (429); Fatty Acid Degradation Pathway (82935); Fatty Acid Degradation Pathway (296); Fatty Acid Metabolism Pathway (868084); Fatty Acid Metabolism Pathway (878045); Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (1270010); Metabolic Pathways (132956); Metabolism Pathway (1269956)
ncbi summary :
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. [provided by RefSeq, Jul 2008]
uniprot summary :
ACOX1: 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. Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD); also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning. Belongs to the acyl-CoA oxidase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Oxidoreductase; Lipid Metabolism - alpha-linolenic acid; EC 1.3.3.6; Lipid Metabolism - unsaturated fatty acid biosynthesis; Lipid Metabolism - fatty acid. Chromosomal Location of Human Ortholog: 17q25.1. Cellular Component: nucleoplasm; peroxisomal membrane; peroxisomal matrix; membrane; mitochondrion; intracellular membrane-bound organelle; plasma membrane; nucleolus; peroxisome; nucleus. Molecular Function: palmitoyl-CoA oxidase activity; acyl-CoA dehydrogenase activity; acyl-CoA oxidase activity; FAD binding; protein N-terminus binding; fatty acid binding; receptor binding; PDZ domain binding. Biological Process: peroxisome fission; generation of precursor metabolites and energy; fatty acid beta-oxidation using acyl-CoA oxidase; very-long-chain fatty acid metabolic process; unsaturated fatty acid metabolic process; lipid homeostasis; spermatogenesis; fatty acid oxidation; lipid metabolic process; cellular lipid metabolic process; prostaglandin metabolic process. Disease: Peroxisomal Acyl-coa Oxidase Deficiency