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PNPO Antibody

  • 货号：
    CSB-PA885740LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA885740LA01HU diluted at 1:600 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA885740LA01HU diluted at 1:600 and staining in paraffin-embedded human endometrial cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of Hela cells with CSB-PA885740LA01HU at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) PNPO Polyclonal antibody
  • Uniprot No.：
    Q9NVS9
  • 基因名：
    PNPO
  • 别名：
    EC 1.4.3.5 antibody; FLJ10535 antibody; PDXPO antibody; PNPO antibody; PNPO_HUMAN antibody; Pyridoxal 5' phosphate synthase antibody; pyridoxamine 5' phosphate oxidase antibody; Pyridoxamine phosphate oxidase antibody; Pyridoxamine-phosphate oxidase antibody; pyridoxine 5' phosphate oxidase antibody; Pyridoxine-5'-phosphate oxidase antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Pyridoxine-5\'-phosphate oxidase protein (114-207AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，PNPO Antibody (CSB-PA885740LA01HU)，的标记方式是Non-conjugated。对于PNPO Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA885740LB01HU PNPO Antibody, HRP conjugated ELISA
    FITC CSB-PA885740LC01HU PNPO Antibody, FITC conjugated
    Biotin CSB-PA885740LD01HU PNPO Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:500-1:1000
    IF 1:200-1:500
  • 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 oxidation of either pyridoxine 5'-phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP).
  • 基因功能参考文献：
    1. TGF-beta1-mediated PNPO expression was at least in part through the upregulation of miR-143-3p in epithelial ovarian cancer. PMID: 29238081
    2. data support a pathogenic role of the c.347G>A (p.Arg116Gln) mutation in PNPO deficiency; the later onset of symptoms and the milder epilepsy phenotype of these expand the disease phenotype PMID: 28818555
    3. Exome sequencing revealed that the patient was compound heterozygous for pathogenic mutations [c.546+1G>A (IVS5+1 G>A) and c.620delG (p.G207VfsX215)] in the PNPO gene. PMID: 26535729
    4. One sequence variant, R116Q, a single nucleotide polymorphism that has been reported in the general population, was found to have an effect on PNPO activity. PMID: 24645144
    5. Challenge to the paradigm of exclusive PLP responsiveness in patients with pyridoxal 5'-phosphate oxidase deficiency and underlines the importance of consecutive testing of pyridoxine and PLP in neonates with antiepileptic drug-resistant seizures. PMID: 24658933
    6. Generalized epilepsies implicates susceptibility genes: CHRM3 at 1q43, VRK2 at 2p16.1, ZEB2 at 2q22.3, SCN1A at 2q24.3 and PNPO at 17q21.32. PMID: 22949513
    7. provide evidence that a single arginine residue of the C terminus of pyridoxal 5'-phosphate synthase is responsible for coordinating co-operativity in this elaborate protein machinery PMID: 21283685
    8. Results describe a combined computer and biochemical approach to characterize human pyridoxine 5'-phosphate oxidase (PNPO). PMID: 15182361
    9. several mutations appear to cause epilepsy PMID: 15772097
    10. We examined 8 single nucleotide polymorphisms (SNPs) in PNPO and its 5'-flanking regions in 359 schizophrenia patients and 582 control subjects. PMID: 17851041
    11. Sequencing of the PNPO gene revealed a novel homozygous nonsense mutationt in exon 3 in the neonatal and infantile seizure. PMID: 18485777
    12. PNP oxidase R229W mutation identified in patients with neonatal epileptic encephalopathy sgnificantly affect the catalytic efficiency of the enzyme. PMID: 19759001

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  • 相关疾病：
    Pyridoxine-5'-phosphate oxidase deficiency (PNPOD)
  • 蛋白家族：
    Pyridoxamine 5'-phosphate oxidase family
  • 数据库链接：

    HGNC: 30260

    OMIM: 603287

    KEGG: hsa:55163

    STRING: 9606.ENSP00000225573

    UniGene: Hs.631742