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PHYH Antibody

  • 货号：
    CSB-PA017946GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    O14832
  • 基因名：
    PHYH
  • 别名：
    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
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human PHYH
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    1. 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
    2. 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
    3. Ten novel PHYH mutations found in Refsum disease patients. PMID: 14974078
    4. 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
    5. 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
    6. 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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  • 相关疾病：
    Refsum disease (RD)
  • 亚细胞定位：
    Peroxisome.
  • 蛋白家族：
    PhyH family
  • 组织特异性：
    Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.
  • 数据库链接：

    HGNC: 8940

    OMIM: 266500

    KEGG: hsa:5264

    STRING: 9606.ENSP00000263038

    UniGene: Hs.498732