Recombinant人UROS protein (ab107136)

概述

  • 产品名称Recombinant人UROS protein
  • 蛋白长度Full length protein

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionP10746
    • 种属Human
    • 序列MGSSHHHHHHSSGLVPRGSHMKVLLLKDAKEDDCGQDPYIRELGLYGLEA TLIPVLSFEFLSLPSFSEKLSHPEDYGGLIFTSPRAVEAAELCLEQNNKT EVWERSLKEKWNAKSVYVVGNATASLVSKIGLDTEGETCGNAEKLAEYIC SRESSALPLLFPCGNLKREILPKALKDKGIAMESITVYQTVAHPGIQGNL NSYYSQQGVPASITFFSPSGLTYSLKHIQELSGDNIDQIKFAAIGPTTAR ALAAQGLPVSCTAESPTPQALATGIRKALQPHGCC
    • 分子量31 kDa including tags
    • 氨基酸1 to 265
    • 标签His tag N-Terminus

技术指标

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.

  • 应用

    Mass Spectrometry

    SDS-PAGE

  • 质谱法
    MALDI-TOF
  • 纯度> 95 % SDS-PAGE.
    ab107136 is purified using conventional chromatography techniques.
  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 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

Recombinant Human UROS protein 图像

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

Recombinant Human UROS protein (ab107136)参考文献

ab107136 has not yet been referenced specifically in any publications.

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