Overview

  • Product name
  • Description
    Rabbit polyclonal to HMBS/PBGD
  • Host species
    Rabbit
  • Tested applications
    Suitable for: IP, WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Rabbit, Horse, Cat, Dog, Pig, Chimpanzee, Rhesus monkey, Gorilla, Orangutan
  • Immunogen

    Synthetic peptide within Human HMBS/PBGD aa 311-361. The exact sequence is proprietary. NP_000181.2
    Sequence:

    DDPQLVGITA RNIPRGPQLA AQNLGISLAN LLLSKGAKNI LDVARQLNDA


    Database link: P08397

  • Positive control
    • HeLa, 293T and Jurkat cell lysates
  • General notes

     This product was previously labelled as HMBS

     

Properties

Applications

Our Abpromise guarantee covers the use of ab185929 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
IP Use at 2-10 µg/mg of lysate.
WB 1/2000 - 1/10000. 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

  • Western blot analysis of Immunoprecipitates.

    Immunoprecipitation of 293T whole cell lysate (1 mg for IP; 20% of IP loaded) using ab185929 at 6 µg/mg lysate for IP and at 1 µg/ml for blotting

    Detection: Chemiluminescence with an exposure time of 10 seconds.

  • All lanes : Anti-HMBS/PBGD antibody (ab185929) at 0.1 µg/ml

    Lane 1 : HeLa whole cell lysate
    Lane 2 : 293T whole cell lysate
    Lane 3 : Jurkat whole cell lysate

    Lysates/proteins at 50 µg per lane.

    Developed using the ECL technique.

    Predicted band size: 39 kDa


    Exposure time: 3 minutes

References

ab185929 has not yet been referenced specifically in any publications.

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