Recombinant Human HMBS protein (ab114899)

Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP08397
    • SpeciesHuman
    • SequenceMSGNGNAAATAEENSPKMRVIRVGTRKSQLARIQTDSVVATLKASYPGLQ FEIIAMSTTGDKIPDTALSKIGEKSLFTKELEHALEKNEVDLVVHSLKDL PTVLPPGFTIGAICKRENPHDAVVFHPKFVGKTLETLPEKSVVGTSSLRR AAQLQRKFPHLEFRSIRGNLNTRLRKLDEQQEFSAIILATAGLQRMGWHN RVGQILHPEECMYAVGQGALGVEVRAKDQDILDLVGVLHDPETLLRCIAE RAFLRHLEGGCSVPVAVHTAMKDGQLYLTGGVWSLDGSDSIQETMQATIH VPAQHEDGPEDDPQLVGITARNIPRGPQLAAQNLGISLANLLLSKGAKNI LDVARQLNDAH
    • Molecular weight66 kDa including tags
    • Amino acids1 to 361

Specifications

Our Abpromise guarantee covers the use of ab114899 in the following tested applications.

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

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

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
  • FunctionTetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
  • Tissue specificityIsoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
  • PathwayPorphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the HMBS family.
  • Cellular localizationCytoplasm.
  • Information by UniProt

Recombinant Human HMBS protein images

  • 12.5% SDS-PAGE showing ab114899 at approximately 65.82kDa.
    Stained with Coomassie Blue.

References for Recombinant Human HMBS protein (ab114899)

ab114899 has not yet been referenced specifically in any publications.

Product Wall

Unfortunately, we have not tested the reactivity of this product and therefore we don’t know if it is an intermediate or as a holo (apo-) enzyme. We have only tested this antibody in WB, SDS-PAGE and ELISA.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"