Product Datasheet  
HMBS Antibody  
Catalog Number: 49010  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:P08397
  • Form of Antibody：  
  • 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • recombinant protein
  • reactivity：  
  • Hu, Ms, Rt
  • appl_detail：  
  • WB: 1:1,000-1:2,000



  • other_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 u
  • Purification：  
  • ProA affinity purified
  • Specificity：  
  • Applications：  
  • WB
  • 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.



 
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