Application
Immunofluorescence staining of formalin-fixed Hep G2 cells showing cytoplasmic localization.Application
Western blot analysis of PBGD expression in U-937 (A) and Hep G2 (B) whole cell lysates.Application
Western blot analysis of PBGD expression in non-transfected: (A) and mouse PBGD transfected: (B) 293T whole cell lysates.Application
Western blot analysis of PBGD expression in Hep G2 whole cell lysate.| Product Name | PBGD Antibody (H-300) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 62-361 mapping at the C-terminus of PBGD of human origin |
| Modification Notes | 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. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of PBGD of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 62-361 mapping at the C-terminus of PBGD of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 62-361 mapping at the C-terminus of PBGD of human origin |
| Entrez Gene | 3145 |
|---|
Application
Immunofluorescence staining of formalin-fixed Hep G2 cells showing cytoplasmic localization.
Application
Western blot analysis of PBGD expression in U-937 (A) and Hep G2 (B) whole cell lysates.
Application
Western blot analysis of PBGD expression in non-transfected: (A) and mouse PBGD transfected: (B) 293T whole cell lysates.
Application
Western blot analysis of PBGD expression in Hep G2 whole cell lysate.| Application Notes | recommended for detection of PBGD of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
