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©2020 by Bon Opus Biosciences, LLC.

HMBS Recombinant Rabbit monoclonal Antibody IgG

SKU: BA111699-100µl
$279.00Price

Fig1: Western blot analysis of HMBS on different lysates using anti-HMBS antibody at 1/1,000 dilution.

Positive control:   

Lane 1: Hela                  

Lane 2: 293T

 

 

Bon Opus Cat. #BA111699
Size
  • Host Species; Species Reactivity

    Rabbit; Human, Mouse, Rat
  • Immunogen

    Recombinant protein
  • Application Summary

    WB
  • Purification; Formulation

    ProA affinity purified; 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.; Liquid form.
  • ALTnames

    Porphobilinogen deaminase, Hydroxymethylbilane synthase, Pre-uroporphyrinogen synthase
  • Background

    PBGD (porphobilinogen deaminase), also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP 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.(ET1610-91)