s10488-016-0725-6

Recombinant Human HMBS Protein Summary

    Description
    A recombinant protein with a N-Terminal His-tag and corresponding to the amino acids 1-361 of Human HMBS

    Source: E.coli

    Amino Acid 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

    Source
    E. coli
    Protein/Peptide Type
    Recombinant Protein
    Gene
    HMBS
    Purity
    >95% pure by SDS-PAGE

Applications/Dilutions

    Theoretical MW
    41.9 kDa.
    Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Packaging, Storage & Formulations

    Storage
    Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
    Buffer
    20mM Tris-HCl buffer (pH 8.0) containing 1mM DTT, 10% glycerol, 0.1M NaCl
    Preservative
    No Preservative
    Concentration
    1 mg/ml
    Purity
    >95% pure by SDS-PAGE

Alternate Names for Recombinant Human HMBS Protein

      hydroxymethylbilane synthasePre-uroporphyrinogen synthase
      PBGDPBG-D
      PORC
      porphobilinogen deaminase
      porphyria, acute; Chester type
      UPSEC 2.5.1.61
      uroporphyrinogen I synthase
      uroporphyrinogen I synthetase

Background

Porphobilinogen deaminase, also known as HMBS, is a member of the hydroxymethylbilane synthase superfamily. It is a cytoplasmic enzyme found in the heme synthesis pathway. Deficiency of HMBS causes errors in pyrrole metabolism which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP) which is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine. Recombinant human HMBS protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Peptides and proteins are guaranteed for 3 months from date of receipt.

product targets : TGF-beta_Smad inhibitors

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