製品の概要

  • 製品名Anti-UROS antibody
    UROS 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal to UROS
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

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

製品の特性

  • 製品の状態Liquid
  • 保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • バッファーPreservative: None
    PBS, pH 7.2
  • Concentration information loading...
  • 精製度Protein G purified
  • ポリ/モノモノクローナル
  • アイソタイプIgG1
  • 軽鎖の種類kappa
  • 研究分野

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB
  • 追加情報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.
  • ターゲット情報

    • 機能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).
    • 組織特異性Ubiquitous.
    • パスウェイPorphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 3/4.
    • 関連疾患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.
    • 配列類似性Belongs to the uroporphyrinogen-III synthase family.
    • Information by UniProt
    • 参照データベース
    • 別名
      • 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 画像



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

    Anti-UROS antibody (ab58097) 使用論文

    ab58097 has not yet been referenced specifically in any publications.

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    There are currently no Abreviews or Questions for ab58097.
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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"