PBGD Antibody (E-9): sc-166743
- PBGD Antibody (E-9) is a mouse monoclonal IgG2a κ PBGD antibody provided at 200 µg/ml
- raised against amino acids 62-361 mapping at the C-terminus of PBGD of human origin
- PBGD Antibody (E-9) is recommended for detection of PBGD of mouse, rat and human origin by WB, IP, IF and ELISA
- Anti-PBGD Antibody (E-9) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG Fc BP-HRP, m-IgG2a BP-HRP and m-IgGκ BP-HRP are the preferred secondary detection reagents for PBGD Antibody (E-9) for WB applications. These reagents are now offered in bundles with PBGD Antibody (E-9) (see ordering information below).
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PBGD Antibody (E-9) is a mouse monoclonal IgG2a kappa light chain antibody that detects PBGD protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). PBGD (E-9) antibody is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. PBGD protein, also known as porphobilinogen deaminase or hydroxymethylbilane synthase, plays a crucial role in the heme biosynthesis pathway, catalyzing the conversion of porphobilinogen to hydroxymethylbilane, which is essential for the production of heme, a vital component of hemoglobin and various enzymes. Proper functioning of PBGD is critical, as deficiencies in this enzyme can lead to acute intermittent porphyria (AIP), a serious inherited disorder characterized by neurological dysfunctions, hypertension, tachycardia, and abdominal pain, along with elevated levels of aminolevulinic acid and porphobilinogen in the urine. PBGD (E-9) monoclonal antibody allows researchers to investigate the role of this enzyme in both normal physiology and disease states, providing valuable insights into the mechanisms underlying porphyrin metabolism and its associated disorders.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
PBGD Antibody (E-9) References:
- Acute intermittent porphyria: expression of mutant and wild-type porphobilinogen deaminase in COS-1 cells. | Mustajoki, S., et al. 2000. Mol Med. 6: 670-9. PMID: 11055586
- Clinical and biochemical characteristics and genotype-phenotype correlation in 143 Finnish and Russian patients with acute intermittent porphyria. | Fraunberg, MVUZ., et al. 2005. Medicine (Baltimore). 84: 35-47. PMID: 15643298
- Mutation hotspots in the human porphobilinogen deaminase gene: recurrent mutations G111R and R173Q occurring at CpG motifs. | Schneider-Yin, X., et al. 2004. J Inherit Metab Dis. 27: 625-31. PMID: 15669678
- Standardization strategy for quantitative PCR in human seminoma and normal testis. | Neuvians, TP., et al. 2005. J Biotechnol. 117: 163-71. PMID: 15823405
- Anesthesia in a child with homozygous porphobilinogen deaminase deficiency: a severe form of acute intermittent porphyria. | Sheppard, L. and Dorman, T. 2005. Paediatr Anaesth. 15: 426-8. PMID: 15828996
- Tissue-specific expression of porphobilinogen deaminase. Two isoenzymes from a single gene. | Grandchamp, B., et al. 1987. Eur J Biochem. 162: 105-10. PMID: 3816774
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
PBGD Antibody (E-9) | sc-166743 | 200 µg/ml | $322.00 | |||
PBGD Antibody (E-9): m-IgG Fc BP-HRP Bundle | sc-529039 | 200 µg Ab; 10 µg BP | $361.00 | |||
PBGD Antibody (E-9): m-IgGκ BP-HRP Bundle | sc-521729 | 200 µg Ab, 40 µg BP | $361.00 | |||
PBGD Antibody (E-9): m-IgG2a BP-HRP Bundle | sc-547242 | 200 µg Ab; 10 µg BP | $361.00 | |||
PBGD Antibody (E-9) AC | sc-166743 AC | 500 µg/ml, 25% agarose | $424.00 | |||
PBGD Antibody (E-9) HRP | sc-166743 HRP | 200 µg/ml | $322.00 | |||
PBGD Antibody (E-9) FITC | sc-166743 FITC | 200 µg/ml | $336.00 | |||
PBGD Antibody (E-9) PE | sc-166743 PE | 200 µg/ml | $349.00 | |||
PBGD Antibody (E-9) Alexa Fluor® 488 | sc-166743 AF488 | 200 µg/ml | $364.00 | |||
PBGD Antibody (E-9) Alexa Fluor® 546 | sc-166743 AF546 | 200 µg/ml | $364.00 | |||
PBGD Antibody (E-9) Alexa Fluor® 594 | sc-166743 AF594 | 200 µg/ml | $364.00 | |||
PBGD Antibody (E-9) Alexa Fluor® 647 | sc-166743 AF647 | 200 µg/ml | $364.00 | |||
PBGD Antibody (E-9) Alexa Fluor® 680 | sc-166743 AF680 | 200 µg/ml | $364.00 | |||
PBGD Antibody (E-9) Alexa Fluor® 790 | sc-166743 AF790 | 200 µg/ml | $364.00 |