ACOX1 Antibody
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货号:CSB-PA001172GA01HU
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规格:¥3,900
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其他:
产品详情
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Uniprot No.:Q15067
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基因名:
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别名:ACOX antibody; ACOX1 antibody; ACOX1_HUMAN antibody; Acyl CoA oxidase 1 palmitoyl antibody; Acyl CoA oxidase straight chain antibody; AOX antibody; EC 1.3.3.6 antibody; PALMCOX antibody; Palmitoyl CoA oxidase antibody; Palmitoyl-CoA oxidase antibody; Peroxisomal acyl coenzyme A oxidase 1 antibody; Peroxisomal acyl-coenzyme A oxidase 1 antibody; Peroxisomal fatty acyl CoA oxidase antibody; SCOX antibody; Straight chain acyl CoA oxidase antibody; Straight-chain acyl-CoA oxidase antibody
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宿主:Rabbit
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反应种属:Human,Mouse,Rat
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免疫原:Human AOX
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免疫原种属:Homo sapiens (Human)
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抗体亚型:IgG
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纯化方式:Antigen Affinity Purified
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浓度:It differs from different batches. Please contact us to confirm it.
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保存缓冲液:PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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产品提供形式:Liquid
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应用范围:ELISA,WB,IHC
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Protocols:
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储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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货期:Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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靶点详情
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功能:Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. First enzyme of the fatty acid beta-oxidation pathway.; Shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length.; Is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl-CoA.
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基因功能参考文献:
- Because patients with AOx deficiency suffer from more severe symptoms than those with X-ALD, accumulation of VLC-PUFA and/or reduction of DHA may be associated with the severity of peroxisomal diseases. PMID: 24418004
- ACOX1 and GNPAT silencing up-regulated ceramide galactosyltransferase (UGT8) mRNA expression, and down-regulated UDP-glucoseceramide glucosyltransferase (UGCG). PMID: 23933200
- Data show that human ACOX1b isoform is more effective than the ACOX1a isoform in reversing the Acox1 null phenotype in the mouse. PMID: 20195242
- Mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency. PMID: 17458872
- report on two new patients with ACOX1 deficiency and mutational analyses PMID: 18536048
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相关疾病:Adrenoleukodystrophy, pseudoneonatal (Pseudo-NALD)
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亚细胞定位:Peroxisome.
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蛋白家族:Acyl-CoA oxidase family
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组织特异性:Widely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Level
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数据库链接:
HGNC: 119
OMIM: 264470
KEGG: hsa:51
STRING: 9606.ENSP00000293217
UniGene: Hs.464137
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