SKU-Pack Size | Availability | Size | Price | |
EAB13574-30UL | In Stock | 30ul | €115.70 | |
EAB13574-100UL | In Stock | 100ul | €258.70 | |
EAB13574-200UL | In Stock | 200ul | €440.70 |
Please select the country you are in to find your local distributor. |
![]() |
Dianost | Phone£º+49 (0) 40 65472-053 |
Bullenkoppel 11£¬ 23896 Poggensee, | E-mail£ºola@olasciences.com | |
Germany | Germany | Web£ºwww.olasciences.com |
Product Information | |
Applications | WB, ELISA |
Species Reactivity | Human, Mouse, Rat |
Host / Isotype | Rabbit IgG |
Clonality | Polyclonal |
Applications Dilutions | WB=1:500-2000 |
MW (kDa) | 40 |
Conjugate | Unconjugate |
Specificity | HMBS/PBGD Rabbit Polyclonal 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 IHC-P-Immunohistochemistry-Paraffin |
|
Reactivity Key H-Human M-Mouse R-Rat Mk-Monkey B-Bovine Pg-Pig Hm-Hamster Dg-Dog C-Chicken X-Xenopus Z-Zebrafish |
Product Bioinformatics | |
Synonym(s) | Hydroxymethylbilane synthase; Porphobilinogen deaminase; UPS; PBGD; PORC; ENCEP; PBG-D; LENCEP; HMBS |
Gene Aliases | HMBS |
UniProt ID | |
Entrez Gene ID |
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 | |
![]() Western blot analysis of extracts on Rat brain cells, using HMBS/PBGD Rabbit Polyclonal Antibody (EAB13574) at 1:1000 dilution. Secondary antibody Goat Anti-Rabbit IgG (H&L)-HRP (EAB21002) at 1:5000 dilution.
|
Specific Protocols | |