Overview

  • Product name

    Recombinant Human UROS protein
  • Protein length

    Full length protein

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MGSSHHHHHHSSGLVPRGSHMKVLLLKDAKEDDCGQDPYIRELGLYGLEA TLIPVLSFEFLSLPSFSEKLSHPEDYGGLIFTSPRAVEAAELCLEQNNKT EVWERSLKEKWNAKSVYVVGNATASLVSKIGLDTEGETCGNAEKLAEYIC SRESSALPLLFPCGNLKREILPKALKDKGIAMESITVYQTVAHPGIQGNL NSYYSQQGVPASITFFSPSGLTYSLKHIQELSGDNIDQIKFAAIGPTTAR ALAAQGLPVSCTAESPTPQALATGIRKALQPHGCC
    • Molecular weight

      31 kDa including tags
    • Amino acids

      1 to 265
    • Tags

      His tag N-Terminus

Specifications

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.

  • Applications

    Mass Spectrometry

    SDS-PAGE

  • Mass spectrometry

    MALDI-TOF
  • Purity

    > 95 % SDS-PAGE.
    ab107136 is purified using conventional chromatography techniques.
  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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.

    pH: 8.00
    Constituents: 0.316% Tris HCl, 10% Glycerol, 0.58% Sodium chloride

General Info

  • Alternative names

    • 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
  • Function

    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).
  • Tissue specificity

    Ubiquitous.
  • Pathway

    Porphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 3/4.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the uroporphyrinogen-III synthase family.
  • Information by UniProt

Images

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

References

ab107136 has not yet been referenced specifically in any publications.

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