概述

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

    Synthetic peptide selected from the N terminal region of Human UROS, conjugated to KLH (NP_000366).

  • 阳性对照
    • K562 cell lysate; Mouse kidney tissue lysate

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at 4°C (up to 6 months). Store at -20°C long term.
  • 存储溶液Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 纯化说明This antibody is purified through a protein A column, followed by peptide affinity purification.
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab95082 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 1/100 - 1/500. Predicted molecular weight: 29 kDa.

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

  • All lanes : Anti-UROS antibody (ab95082) at 1/100 dilution

    Lane 1 : K562 cell lysate
    Lane 2 : Mouse kidney tissue lysate

    Lysates/proteins at 35 µg per lane.


    Predicted band size : 29 kDa

Anti-UROS antibody (ab95082)参考文献

ab95082 has not yet been referenced specifically in any publications.

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