Overview

  • Product name
    Anti-HMBS/PBGD antibody [EPR8105]
    See all HMBS/PBGD primary antibodies
  • Description
    Rabbit monoclonal [EPR8105] to HMBS/PBGD
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WBmore details
    Unsuitable for: Flow Cyt,ICC/IF,IHC-P or IP
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    corresponding to Human HMBS/PBGD aa 300-400.
    Database link: P08397

  • Positive control
    • HepG2, HeLa, U937, and K562 cell lysates
  • General notes

     

     

     This product was previously labelled as HMBS

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab129092 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 1/1000 - 1/10000. Detects a band of approximately 42-44 kDa (predicted molecular weight: 39 kDa).Can be blocked with Recombinant Human HMBS/PBGD protein (ab123176).
  • Application notes
    Is unsuitable for Flow Cyt,ICC/IF,IHC-P or IP.
  • 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

    • All lanes : Anti-HMBS/PBGD antibody [EPR8105] (ab129092) at 1/1000 dilution

      Lane 1 : HepG2 cell lysates
      Lane 2 : HeLa cell lysates
      Lane 3 : U937 cell lysates
      Lane 4 : K562 cell lysates

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution

      Predicted band size: 39 kDa
      Observed band size: 42-44 kDa
      why is the actual band size different from the predicted?

    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD

    References

    This product has been referenced in:
    • Chen B  et al. Identification and characterization of 40 novel hydroxymethylbilane synthase mutations that cause acute intermittent porphyria. J Inherit Metab Dis 42:186-194 (2019). Read more (PubMed: 30740734) »
    See 1 Publication for this product

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    Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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