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PPOX Antibody

  • 货号：
    CSB-PA018502GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P50336
  • 基因名：
    PPOX
  • 别名：
    MGC8485 antibody; PPO antibody; PPOX antibody; PPOX_HUMAN antibody; Protoporphyrinogen oxidase antibody; V290M antibody; Variegate porphyria antibody; VP antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human PPOX
  • 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 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX.
  • 基因功能参考文献：
    1. the regulation of PPOX gene expression can also occur through a post-transcriptional modulation of the amount of gene product and this modulation can be mediated by 5' untranslated exon 1. PMID: 27667166
    2. GAPDH and protoporphyrinogen oxidase were shown to have higher expression in faster growing cell lines and primary tumors. Pharmacologic inhibition of GAPDH or PPOX reduced the growth of colon cancer cells in vitro PMID: 25944804
    3. Variegate porphyria is the result of decreased protoporphyrinogen oxidase activity. Diet supplementation with vitamins E and C restores PPOX gene expression in lymphocytes of variegate porphyria patients. PMID: 23601071
    4. in the hepatic cancer tissue of two acute porphyria patients, somatic second-hit mutations result in nearly complete inactivation of PPOX and HMBS PMID: 25445397
    5. The c.851G>T and the c.1013C>G of PPOX gene were found in two and four unrelated families respectively. 1 experienced only photosensitivity, 1 only neurological symptoms and the 2 both clinical manifestations. PMID: 19656455
    6. The mutation 1082-1083insC(1 base pair insertion at position 1082) in exon 10 of the PPOX gene was prevalent in the Swiss population. PMID: 19656457
    7. findings emphasize the usefulness of MLPA analysis as a complement to PPOX gene sequencing analysis for comprehensive genetic diagnostics in patients with variegate prophyria PMID: 23324528
    8. VP-causing mutation affect the catalytic activity of hPPO by affecting the ability of hPPO to sample the privileged conformations PMID: 23467411
    9. sequenced 27 members of a family with variegate porphyria with a T>A transversion at the intron 6 consensus splicing site in 8 patients and 4 SNPs: c.-414A>C; IVS2+121G>C; c.1188G>A and IVS12+34C>T PMID: 19229653
    10. data deliver further confirmation that the South African and Dutch variegate porphyria families carrying mutation p.R59W shared a common ancestor. PMID: 21910705
    11. Forty-seven variegate porphyria-causing mutations were purified by chromatography and kinetically characterized in vitro. PMID: 21048046
    12. Two previously undescribed mutations were identified: PPOX1423-1426-delATCT and PPOX2272insG. PMID: 12017191
    13. This enzyme in its regular and mutagenized forms is targeted to mitochondria when transfected. PMID: 12556518
    14. Information required for targeting to the mitochondria is contained within the first 250 amino acid residues of human PPOX. PMID: 14535846
    15. Modulation of penetrance by the wild-type allele in dominantly inherited erythrohepatic and acute hepatic porphyrias was studied using PPOX. PMID: 14669009
    16. Protoporphyrinogen oxidase targeting mechanism to the mitochondrion. PMID: 16621625
    17. Mutation in protoporphyrinogen oxidase is associated with variegate porphyria PMID: 16947091
    18. GATA-1 binding sites in exon 1 constitute key regulatory elements in differential expression of PPOX in erythroid and non-erythroid cells. PMID: 18191920

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  • 相关疾病：
    Variegate porphyria (VP)
  • 亚细胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side.
  • 蛋白家族：
    Protoporphyrinogen oxidase family
  • 组织特异性：
    Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • 数据库链接：

    HGNC: 9280

    OMIM: 176200

    KEGG: hsa:5498

    STRING: 9606.ENSP00000343943

    UniGene: Hs.517373