Overview

  • Product name

  • Description

    Rabbit polyclonal to HMBS/PBGD
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IF, IHC-P, WBmore details
  • Species reactivity

    Reacts with: Human, Pig
    Predicted to work with: Mouse, Rat, Cow
  • Immunogen

    Recombinant fragment (His-T7-tag) corresponding to Human HMBS/PBGD aa 85-337. (Expressed in E.coli).
    Sequence:

    LEKNEVDLVVHSLKDLPTVLPPGFTIGAICKRENPHDAVVFHPKFVGKTL ETLPEKSVVGTSSLRRAAQLQRKFPHLEFRSIRGNLNTRLRKLDEQQEFS AIILATAGLQRMGWHNRVGQILHPEECMYAVGQGALGVEVRAKDQDILDL VGVLHDPETLLRCIAERAFLRHLEGGCSVPVAVHTAMKDGQLYLTGGVWS LDGSDSIQETMQATIHVPAQHEDGPEDDPQLVGITARNIPRGPQLAAQNL GIS


    Database link: P08397

  • Positive control

    • WB: Recombinant human HMBS/PBGD protein; HeLa, HEK-293T and HepG2 cell lysates; Pig heart lysate. IHC-P: Human pancreatic cancer tissue. ICC/IF: HeLa cells.
  • General notes

     This product was previously labelled as HMBS

     

Properties

Applications

Our Abpromise guarantee covers the use of ab232927 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
ICC/IF Use a concentration of 5 - 20 µg/ml.
IHC-P Use a concentration of 5 - 20 µg/ml.
WB Use a concentration of 0.5 - 2 µg/ml. Predicted molecular weight: 39 kDa.

Target

  • Function

    Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
  • Tissue specificity

    Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
  • Pathway

    Porphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4.
  • Involvement in disease

    Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. 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. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
  • Sequence similarities

    Belongs to the HMBS family.
  • Cellular localization

    Cytoplasm.
  • Information by UniProt
  • Database links

  • Alternative names

    • HEM3_HUMAN antibody
    • HMBS antibody
    • Hydroxymethylbilane synthase antibody
    • PBG D antibody
    • PBG-D antibody
    • PBGD antibody
    • PORC antibody
    • Porphobilinogen deaminase antibody
    • porphyria, acute; Chester type antibody
    • Pre uroporphyrinogen synthase antibody
    • Pre-uroporphyrinogen synthase antibody
    • UPS antibody
    • Uroporphyrinogen I synthase antibody
    • Uroporphyrinogen I synthetase antibody
    see all

Images

  • Anti-HMBS/PBGD antibody (ab232927) at 2 µg/ml + Recombinant human HMBS/PBGD protein

    Secondary
    HRP-Linked Guinea pig anti-Rabbit at 1/2000 dilution

    Predicted band size: 39 kDa

  • All lanes : Anti-HMBS/PBGD antibody (ab232927) at 2 µg/ml

    Lane 1 : HeLa (human epithelial cell line from cervix adenocarcinoma) cell lysate
    Lane 2 : HEK-293T (human epithelial cell line from embryonic kidney transformed with large T antigen) cell lysate
    Lane 3 : HepG2 (human liver hepatocellular carcinoma cell line) cell lysate
    Lane 4 : Pig heart lysate

    Secondary
    All lanes : HRP-Linked Guinea pig anti-Rabbit at 1/2000 dilution

    Predicted band size: 39 kDa

  • Paraffin-embedded human pancreatic cancer tissue stained for HMBS/PBGD using ab232927 at 10 μg/ml in immunohistochemical analysis. DAB staining.

  • HeLa (human epithelial cell line from cervix adenocarcinoma) cells stained for HMBS/PBGD (green) using ab232927 at 20 μg/ml in ICC/IF, followed by FITC staining.

References

ab232927 has not yet been referenced specifically in any publications.

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