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HMBS/PBGD Rabbit Monoclonal Antibody
Catalog #£ºEAB21669
  SKU-Pack Size Availability Size Price
EAB21669-100UL In Stock 100ul €297.70
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SBT BioChem Srl Phone£º+39 05 7156 8147
Via Ponte di Bagnolo, 811 Bologna, E-mail£ºinfo@sbtbiochem.it
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Product Information
Applications WB
Species Reactivity Human, Mouse, Rat
Host / Isotype Rabbit IgG
Clonality Monoclonal
Applications Dilutions WB=1:500-2000¡¡
MW (kDa) 40
Conjugate Unconjugate
Specificity HMBS/PBGD Rabbit Monoclonal Antibody detects endogenous levels of HMBS/PBGD protein.
Purification Affinity purification
Concentration 1mg/ml
Format Liquid
Formulation In PBS, pH 7.4, containing 0.02% sodium azide,0.5% BSA and 50% glycerol.
Shipping Gel Pack
Storage Store at -20¡ãC least 1 year from the date of shipment. avoid repeated freeze/thaw cycles. Aliquots may be stored at +4¡ãC for 1-2 weeks.
Research Use For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.
Application Key

WB-Western Blot IP-Immunoprecipitation IHC-Immunohistochemistry IF-Immunofluorescence ICC-Immunocytochemistry FC-Flow Cytometry

Reactivity Key

H-Human M-Mouse R-Rat Mk-Monkey B-Bovine Pg-Pig Hm-Hamster Dg-Dog C-Chicken X-Xenopus Z-Zebrafish Hr-Horse All-All Species Expected

Product Bioinformatics
Synonym(s) Hydroxymethylbilane synthase; Porphobilinogen deaminase; UPS; PBGD; PORC; ENCEP; PBG-D; LENCEP; HMBS
Gene Aliases HMBS
UniProt ID

P08397 

Entrez Gene ID

3145 

Product Description

HMBS also known as PBGD is a member of the hydroxymethylbilane synthase superfamily. PBGD plays a crucial role in the heme synthesis pathway, catalyzing the conversion of porphobilinogen into hydroxymethylbilane, which is a key intermediate in the production of heme, an essential component of hemoglobin and various enzymes. The proper functioning of PBGD is vital, as deficiencies in this enzyme can lead to acute intermittent porphyria (AIP), a serious inherited disorder characterized by neurological dysfunctions, abdominal pain, and elevated levels of porphobilinogen and aminolevulinic acid in the urine. Understanding the structure and function of PBGD is important not only for diagnosing and managing AIP but also for exploring potential therapeutic interventions that could mitigate the effects of this disorder.

Product Image Gallery
Specific Protocols
>> Western Blotting Protocol >> Immunoprecipitation Protocol
>> Immunohistochemistry Protocol >> Immunofluorescence Protocol
>> Immunocytochemistry Protocol >> Flow Cytometry Protocol
>> ChIP Protocol >> ELISA Protocol
>> HPLC Protocol >> PCR Protocol
For Research Use Only, Not For Diagnostic Or Therapeutic Procedures.
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