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CPOX Antibody

  • 货号：
    CSB-PA005910GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P36551
  • 基因名：
    CPOX
  • 别名：
    Coprogen oxidase antibody; COPROPORPHYRIA antibody; Coproporphyrinogen III oxidase antibody; Coproporphyrinogen Oxidase (CPOX) antibody; Coproporphyrinogen-III oxidase antibody; Coproporphyrinogenase antibody; COX antibody; CPO antibody; Cpox antibody; CPX antibody; HEM 6 antibody; Hem-6 antibody; Hem6 antibody; HEM6_HUMAN antibody; mitochondrial antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human CPOX
  • 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：
    Involved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.
  • 基因功能参考文献：
    1. The monomer form of mutated CPOX did not show any activity and homodimeric enzymes derived from Hereditary coproporphyria (HCP) mutant showed low activity (<20% of the control). PMID: 24078084
    2. Polymorphism of coproporphyrinogen oxidase is associated with genetic susceptibility to the adverse neurobehavioral effects of Hg exposure in children. PMID: 22765978
    3. Deletion of the fifth exon in the CPOX gene is associated with hereditary coproporphyria. PMID: 21231929
    4. CPOX polymorphisms are associated with biological media contamination and apoptosis disorders. PMID: 22288185
    5. competitive action of both uroporphyrinogen decarboxylase and CPO on the same diacetate porphyrinogen substrate provides additional perspectives on the potential existence of abnormal pathways for heme biosynthesis PMID: 21277781
    6. disease-producing mutations in the CPO gene in nine Swedish families with hereditary coproporphyria PMID: 12181641
    7. coproporphyrinogen III oxidase sequence matches many structural features from urate oxidase PMID: 12208494
    8. Modulation of penetrance by the wild-type allele in dominantly inherited erythrohepatic and acute hepatic porphyrias was studied using CPO. PMID: 14669009
    9. All other type of mutations or missense mutations mapped elsewhere throughout the CPO gene, lead to coproporphyrin accumulation and subsequently typical HCP. PMID: 16159891
    10. CPO mutations form the structural basis of hereditary coproporphyria. PMID: 16176984
    11. The Km recognition) and Kcat values for coproporphyrinogen III and IV were determined. PMID: 16258391
    12. His158 of human CPO may have a role in the active site, but none of the conserved histidine residues of human coproporphyrinogen oxidase is essential for catalytic activity. PMID: 17179900
    13. The s report the association of a novel mutation in the coproporphyrinogen oxidase gene in an Irish pedigree with the devlopment of hereditary coproporphyria PMID: 18557518
    14. Three of the novel missense mutations and one frameshift mutation was detected in coproporphyrinogen III oxidase (CPO) gene in five Italian patients affected by Hereditary Coproporphyria (HCP). PMID: 19267996
    15. biochemical & kinetic properties of CPOX4, the product of a polymorphism of the CPOX gene that modifies effects of mercury on neurobehavioral function; suggests CPOX4 may predispose to impaired heme biosynthesis which is limited further by Hg exposure PMID: 19339664

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  • 相关疾病：
    Hereditary coproporphyria (HCP)
  • 亚细胞定位：
    Mitochondrion intermembrane space.
  • 蛋白家族：
    Aerobic coproporphyrinogen-III oxidase family
  • 数据库链接：

    HGNC: 2321

    OMIM: 121300

    KEGG: hsa:1371

    STRING: 9606.ENSP00000264193

    UniGene: Hs.476982