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UROS Antibody

  • 货号：
    CSB-PA025679LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) UROS Polyclonal antibody
  • Uniprot No.：
    P10746
  • 基因名：
    UROS
  • 别名：
    congenital erythropoietic porphyria antibody; HEM4_HUMAN antibody; Hydroxymethylbilane hydrolyase [cyclizing] antibody; Hydroxymethylbilane hydrolyase antibody; OTTHUMP00000020709 antibody; OTTHUMP00000020710 antibody; UROIIIS antibody; Uroporphyrinogen III cosynthetase antibody; Uroporphyrinogen III synthase (congenital erythropoietic porphyria) antibody; Uroporphyrinogen III synthase antibody; Uroporphyrinogen-III cosynthase antibody; Uroporphyrinogen-III synthase antibody; UROS antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Uroporphyrinogen-III synthase protein (1-265AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，UROS Antibody (CSB-PA025679LA01HU)，的标记方式是Non-conjugated。对于UROS 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-PA025679LB01HU UROS Antibody, HRP conjugated ELISA
    FITC CSB-PA025679LC01HU UROS Antibody, FITC conjugated
    Biotin CSB-PA025679LD01HU UROS 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
  • 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 cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for the various sub-pathways leading to the wide diversity of porphyrins. Porphyrins act as cofactors for a multitude of enzymes that perform a variety of processes within the cell such as methionine synthesis (vitamin B12) or oxygen transport (heme).
  • 基因功能参考文献：
    1. we show evidence that abnormal protein homeostasis is a prevalent mechanism responsible for UROS deficiency and that modulators of UROS proteolysis such as proteasome inhibitors or chemical chaperones may represent an attractive therapeutic option to reduce porphyrin accumulation and prevent skin photosensitivity in Congenital erythropoietic porphyria (CEP)patients when the genotype includes a missense variant. PMID: 28334762
    2. A, T to C change at nucleotide 34313, leading to a substitution of Leucine by Proline at codon 237, was observed in the homozygous state in a family with congenital erythropoietic porphyria. PMID: 22350154
    3. REVIEW: Structural, thermodynamic, and mechanistical studies in uroporphyrinogen III synthase and molecular basis of congenital erythropoietic porphyria PMID: 21570665
    4. Intracellular rescue of the uroporphyrinogen III synthase activity in enzymes carrying the hotspot mutation C73R. PMID: 21343304
    5. Data show that branchpoint sequence (BPS)mutation reduced the wild-type transcript and UROS enzyme activity in CEP lymphoblasts to approximately 10% and 15% of normal, respectively. PMID: 19965637
    6. two novel misense mutations in the UROS gene. PMID: 17298225
    7. NMR analyses of URO-synthase titrated with competitive inhibitors N(D)-methyl-1-formylbilane or URO'gen III, revealed resonance perturbations of specific residues lining the cleft between 2 major domains of URO synthase that mapped enzyme's active site. PMID: 18004775
    8. The decrease in the expression of ubiquitous HMBS and UROS mRNAs under hypoxia is associated with accumulation of hypoxia-inducible factor 1alpha protein. PMID: 19021769
    9. UROS mutations related to erythropoietic porphyria identify a key helix for protein stability. PMID: 19099412

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  • 相关疾病：
    Congenital erythropoietic porphyria (CEP)
  • 蛋白家族：
    Uroporphyrinogen-III synthase family
  • 组织特异性：
    Ubiquitous.
  • 数据库链接：

    HGNC: 12592

    OMIM: 263700

    KEGG: hsa:7390

    STRING: 9606.ENSP00000357775

    UniGene: Hs.501376