Overview

  • Product name
    Anti-UROS antibody
  • Description
    Rabbit polyclonal to UROS
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    Synthetic peptide selected from the N terminal region of Human UROS, conjugated to KLH (NP_000366).

  • Positive control
    • K562 cell lysate; Mouse kidney tissue lysate

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Store at 4°C (up to 6 months). Store at -20°C long term.
  • Storage buffer
    Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Purification notes
    This antibody is purified through a protein A column, followed by peptide affinity purification.
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab95082 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 1/100 - 1/500. Predicted molecular weight: 29 kDa.

Target

  • 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
  • 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

Images

  • All lanes : Anti-UROS antibody (ab95082) at 1/100 dilution

    Lane 1 : K562 cell lysate
    Lane 2 : Mouse kidney tissue lysate

    Lysates/proteins at 35 µg per lane.


    Predicted band size : 29 kDa

References

ab95082 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"

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