Overview

  • Product nameAnti-UROS antibody
    See all UROS primary antibodies
  • Description
    Mouse monoclonal to UROS
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Recombinant full length protein, corresponding to amino acids 1-266 of Human UROS

Properties

  • FormLiquid
  • Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • Storage bufferPreservative: None
    PBS, pH 7.2
  • Concentration information loading...
  • PurityProtein G purified
  • ClonalityMonoclonal
  • IsotypeIgG1
  • Light chain typekappa
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab58097 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB
  • Application notesWB: Use at a concentration of 1-5 µg/ml.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • Target

    • 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
    • Database links
    • Alternative names
      • 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

    Anti-UROS antibody images



    • Predicted band size : 29 kDa
      UROS antibody (ab58097) at 1ug/lane + HL-60 cell lysate at 25ug/lane.

    References for Anti-UROS antibody (ab58097)

    ab58097 has not yet been referenced specifically in any publications.

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"