製品の概要

  • 製品名
    Anti-UROS antibody
  • 製品の詳細
    Rabbit polyclonal to UROS
  • アプリケーション
    適用あり: WBmore details
  • 種交差性
    交差種: Mouse, Human
  • 免疫原

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

  • ポジティブ・コントロール
    • K562 cell lysate; Mouse kidney tissue lysate

製品の特性

  • 製品の状態
    Liquid
  • 保存方法
    Shipped at 4°C. Store at 4°C (up to 6 months). Store at -20°C long term.
  • バッファー
    Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • 精製度
    Immunogen affinity purified
  • 特記事項(精製)
    This antibody is purified through a protein A column, followed by peptide affinity purification.
  • ポリ/モノ
    ポリクローナル
  • アイソタイプ
    IgG
  • 研究分野

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB 1/100 - 1/500. Predicted molecular weight: 29 kDa.

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

画像

  • 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

プロトコール

参考文献

ab95082 has not yet been referenced specifically in any publications.

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