PHYH Antibody
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中文名稱：PHYH兔多克隆抗體
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貨號：CSB-PA017946GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：PHYH
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別名：LN1 antibody; LNAP1 antibody; LNAP1, mouse, homolog of antibody; OTTHUMP00000019131 antibody; OTTHUMP00000019132 antibody; OTTHUMP00000179083 antibody; OTTHUMP00000216226 antibody; PAHX antibody; PAHX_HUMAN antibody; peroxisomal antibody; PhyH antibody; PHYH1 antibody; Phytanic acid oxidase antibody; phytanoil-CoA alpha hydroxylase antibody; phytanoyl CoA 2 hydroxylase antibody; Phytanoyl CoA 2 oxoglutarate dioxygenase antibody; Phytanoyl CoA alpha hydroxylase antibody; Phytanoyl CoA dioxygenase antibody; Phytanoyl CoA dioxygenase peroxisomal antibody; Phytanoyl-CoA alpha-hydroxylase antibody; Phytanoyl-CoA dioxygenase antibody; RD antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human PHYH
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：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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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Catalyzes the 2-hydroxylation of not only racemic phytanoyl-CoA and the isomers of 3-methylhexadecanoyl-CoA, but also a variety of other mono-branched 3-methylacyl-CoA esters (with a chain length of at least seven carbon atoms) and straight-chain acyl-CoA esters (with a chain length longer than four carbon atoms). Does not hydroxylate long and very long straight chain acyl-CoAs or 2-methyl- and 4-methyl-branched acyl-CoAs.
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基因功能參考文獻：
- 3 heterozygous variants: c.85C>T (p.Pro29Ser), c.135-2A>G, and c.768del63bp (p.Phe257Glnfs*16) were found in a family with Refsum's disease. PMID: 28681609
- substrate specificity of PAHX is broader than expected, so Refsum disease might be characterized by an accumulation of not only phytanic acid but also other 3-alkyl-branched fatty acids PMID: 12923223
- Ten novel PHYH mutations found in Refsum disease patients. PMID: 14974078
- demonstrate that both unprocessed and processed forms are able to hydroxylate a range of CoA derivatives; site-directed mutagenesis was used to support proposals for the identity of the iron binding istes of PAHX PMID: 15930519
- manner in which phytanoyl-CoA 2-hydroxylase (PAHX) binds to iron(II) and 2-oxoglutarate at its active site distinguishes it from that of the other human 2-oxoglutarate (2OG)-dependent oxygenase PMID: 16186124
- In the absence of elevated phytanic acid concentrations, clinical neurologic abnormalities in heterozygous relatives of Refsum patients are not attributable to heterozygosity for PAHX mutations. PMID: 18612766
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相關疾病：Refsum disease (RD)
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亞細胞定位：Peroxisome.
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蛋白家族：PhyH family
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組織特異性：Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.
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數據庫鏈接：
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