製品の概要

  • 製品名Recombinant Human UROS protein
  • タンパク質長Full length protein

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号P10746
    • 生物種Human
    • 配列MGSSHHHHHHSSGLVPRGSHMKVLLLKDAKEDDCGQDPYIRELGLYGLEA TLIPVLSFEFLSLPSFSEKLSHPEDYGGLIFTSPRAVEAAELCLEQNNKT EVWERSLKEKWNAKSVYVVGNATASLVSKIGLDTEGETCGNAEKLAEYIC SRESSALPLLFPCGNLKREILPKALKDKGIAMESITVYQTVAHPGIQGNL NSYYSQQGVPASITFFSPSGLTYSLKHIQELSGDNIDQIKFAAIGPTTAR ALAAQGLPVSCTAESPTPQALATGIRKALQPHGCC
    • 分子量31 kDa including tags
    • 領域1 to 265
    • タグHis tag N-Terminus

特性

Our Abpromise guarantee covers the use of ab107136 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

    Mass Spectrometry

    SDS-PAGE

  • 質量分析
    MALDI-TOF
  • 精製度> 95 % SDS-PAGE.
    ab107136 is purified using conventional chromatography techniques.
  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    Preservative: None
    Constituents: 10% Glycerol, 0.1M Sodium chloride, 20mM Tris HCl, pH 8.0

関連情報

  • 別名
    • congenital erythropoietic porphyria
    • HEM4_HUMAN
    • Hydroxymethylbilane hydrolyase
    • Hydroxymethylbilane hydrolyase [cyclizing]
    • OTTHUMP00000020709
    • OTTHUMP00000020710
    • UROIIIS
    • Uroporphyrinogen III cosynthetase
    • Uroporphyrinogen III synthase
    • Uroporphyrinogen III synthase (congenital erythropoietic porphyria)
    • Uroporphyrinogen-III cosynthase
    • Uroporphyrinogen-III synthase
    • UROS
    see all
  • 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

Recombinant Human UROS protein 画像

  • 15% SDS-PAGE showing ab107136 (3µg).

Recombinant Human UROS protein (ab107136) 使用論文

ab107136 has not yet been referenced specifically in any publications.

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