ACOX1 Antibody, Biotin conjugated (PACO47893)
- SKU:
- PACO47893
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- Biotin
Description
抗体名: | ACOX1 Antibody, Biotin conjugated (PACO47893) |
抗体コード: | PACO47893 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Peroxisomal acyl-coenzyme A oxidase 1 protein (113-273AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Biotin |
バックグラウンド: | 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. |
シノニム: | Peroxisomal acyl-coenzyme A oxidase 1 (AOX) (EC 1.3.3.6) (Palmitoyl-CoA oxidase) (Straight-chain acyl-CoA oxidase) (SCOX), ACOX1, ACOX |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:EC 1.3.3.6; Oxidoreductase; Lipid Metabolism - unsaturated fatty acid biosynthesis; Lipid Metabolism - fatty acid; Lipid Metabolism - alpha-linolenic acid Chromosomal Location of Human Ortholog: 17q25.1 Cellular Component: intracellular membrane-bound organelle; membrane; nucleolus; nucleoplasm; nucleus; peroxisomal matrix; peroxisome; plasma membrane Molecular Function:acyl-CoA binding; acyl-CoA dehydrogenase activity; acyl-CoA oxidase activity; electron carrier activity; PDZ domain binding; protein N-terminus binding; receptor binding Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid oxidation; generation of precursor metabolites and energy; lipid metabolic process; prostaglandin metabolic process; very-long-chain fatty acid metabolic process Disease: Peroxisomal Acyl-coa Oxidase Deficiency |
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 Code: | Q15067 |
NCBI GenInfo Identifier: | 126302511 |
NCBI Gene ID: | 51 |
NCBI Accession: | Q15067.3 |
UniProt Secondary Accession: | Q15067,Q12863, Q15068, Q15101, Q16131, Q7Z3W5, Q9UD31 A8K6X8, A8KAA0, B4DK61, F5GYQ8, |
UniProt Related Accession: | Q15067 |
Molecular Weight: | 70,136 Da |
NCBI Full Name: | Peroxisomal acyl-coenzyme A oxidase 1 |
NCBI Synonym Full Names: | acyl-CoA oxidase 1 |
NCBI Official Symbol: | ACOX1 |
NCBI Official Synonym Symbols: | ACOX; SCOX; PALMCOX |
NCBI Protein Information: | peroxisomal acyl-coenzyme A oxidase 1 |
UniProt Protein Name: | Peroxisomal acyl-coenzyme A oxidase 1 |
UniProt Synonym Protein Names: | Palmitoyl-CoA oxidase; Straight-chain acyl-CoA oxidase; SCOX |
Protein Family: | Peroxisomal acyl-coenzyme A oxidase |
UniProt Gene Name: | ACOX1 |
UniProt Entry Name: | ACOX1_HUMAN |