Overview

  • Product name

    Recombinant Human HMBS/PBGD protein
  • Protein length

    Full length protein

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MGSSHHHHHH SSGLVPRGSH MGSHMSGNGN AAATAEENSP KMRVIRVGTR KSQLARIQTD SVVATLKASY PGLQFEIIAM STTGDKILDT ALSKIGEKSL FTKELEHALE KNEVDLVVHS LKDLPTVLPP GFTIGAICKR ENPHDAVVFH PKFVGKTLET LPEKSVVGTS SLRRAAQLQR KFPHLEFRSI RGNLNTRLRK LDEQQEFSAI ILATAGLQRM GWHNRVGQIL HPEECMYAVG QGALGVEVRA KDQDILDLVG VLHDPETLLR CIAERAFLRH LEGGCSVPVA VHTAMKDGQL YLTGGVWSLD GSDSIQETMQ ATIHVPAQHE DGPEDDPQLV GITARNIPRG PQLAAQNLGI SLANLLLSKG AKNILDVARQ LNDAH
    • Molecular weight

      42 kDa including tags
    • Amino acids

      1 to 361
    • Tags

      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab123176 in the following tested applications.

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

  • Applications

    Mass Spectrometry

    SDS-PAGE

  • Mass spectrometry

    MALDI-TOF
  • Purity

    > 95 % SDS-PAGE.
    ab123176 is purified using conventional chromatography techniques.
  • Form

    Liquid
  • Additional notes

     This product was previously labelled as HMBS

     

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride

General Info

  • Alternative names

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

Images

  • 15% SDS-PAGE analysis of ab123176 (3ug)

References

ab123176 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Answer

PBGD enzyme product cat# ab123176 is purified as holo (or apo) enzyme.

Read More

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