概述

  • 产品名称Anti-UROS抗体
    参阅全部 UROS 一抗
  • 描述
    小鼠单克隆抗体to UROS
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length protein, corresponding to amino acids 1-266 of Human UROS

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: None
    PBS, pH 7.2
  • Concentration information loading...
  • 纯度Protein G purified
  • 克隆单克隆
  • 同种型IgG1
  • 轻链类型kappa
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab58097 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB
  • 应用说明WB: Use at a concentration of 1-5 µg/ml.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 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).
    • 组织特异性Ubiquitous.
    • 通路Porphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 3/4.
    • 疾病相关Defects in UROS are the cause of congenital erythropoietic porphyria (CEP) [MIM:263700]; also known as Gunther disease. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. The manifestations of CEP are heterogeneous, ranging from nonimmune hydrops fetalis due to severe hemolytic anemia in utero to milder, later onset forms, which have only skin lesions due to cutaneous photosensitivity in adult life. The deficiency in UROS activity results in the non-enzymatic conversion of hydroxymethylbilane (HMB) into the uroporphyrinogen-I isomer.
      Note=Severe congenital erythropoietic porphyria is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
    • 序列相似性Belongs to the uroporphyrinogen-III synthase family.
    • Information by UniProt
    • 数据库链接
    • 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
      see all

    Anti-UROS antibody 图像



    • Predicted band size : 29 kDa
      UROS antibody (ab58097) at 1ug/lane + HL-60 cell lysate at 25ug/lane.

    Anti-UROS antibody (ab58097)参考文献

    ab58097 has not yet been referenced specifically in any publications.

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