Overview

  • Product nameRecombinant Human UROS protein
  • Protein lengthFull length protein

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionP10746
    • SpeciesHuman
    • SequenceMGSSHHHHHHSSGLVPRGSHMKVLLLKDAKEDDCGQDPYIRELGLYGLEA TLIPVLSFEFLSLPSFSEKLSHPEDYGGLIFTSPRAVEAAELCLEQNNKT EVWERSLKEKWNAKSVYVVGNATASLVSKIGLDTEGETCGNAEKLAEYIC SRESSALPLLFPCGNLKREILPKALKDKGIAMESITVYQTVAHPGIQGNL NSYYSQQGVPASITFFSPSGLTYSLKHIQELSGDNIDQIKFAAIGPTTAR ALAAQGLPVSCTAESPTPQALATGIRKALQPHGCC
    • Molecular weight31 kDa including tags
    • Amino acids1 to 265
    • TagsHis 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.
  • FormLiquid
  • 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.

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

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
  • FunctionCatalyzes 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 specificityUbiquitous.
  • PathwayPorphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 3/4.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the uroporphyrinogen-III synthase family.
  • Information by UniProt

Recombinant Human UROS protein images

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

References for Recombinant Human UROS protein (ab107136)

ab107136 has not yet been referenced specifically in any publications.

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