Application
Fig1: Western blot analysis of HMBS on different lysates using anti-HMBS antibody at 1/1,000 dilution. Positive control: Lane 1: Hela Lane 2: 293T| Product Name | HMBS [SC65-07] |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | 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 uroporphyrinogen synthase antibody Pre-uroporphyrinogen synthase antibody UPS antibody Uroporphyrinogen I synthase antibody Uroporphyrinogen I synthetase antibody |
| Product description | 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. |
| Immunogen | recombinant protein |
| Clonality | Monoclonal |
|---|---|
| Isotype | IgG |
| Host Species | Recombinant rabbit |
| Tested Applications | WB |
| WB:1:1,000-1:2,000 |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Alternative 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 uroporphyrinogen synthase antibody Pre-uroporphyrinogen synthase antibody UPS antibody Uroporphyrinogen I synthase antibody Uroporphyrinogen I synthetase antibody |
|---|---|
| Molecular Weight(MW) | 40 kDa |
| Cellular Localization | Cytoplasm. |
Application
Fig1: Western blot analysis of HMBS on different lysates using anti-HMBS antibody at 1/1,000 dilution. Positive control: Lane 1: Hela Lane 2: 293T| Positive Control | Hela, 293T. |
|---|---|
| Application Notes | WB:1:1,000-1:2,000 |
| Form | Liquid |
|---|---|
| Storage Instructions | Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles. |
| Storage Buffer | 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide. |
