HMBS Polyclonal Antibody
Applications
Reactivity
| Overview | |
| Catalog # | bs-9068R |
| Product Name | HMBS Polyclonal Antibody |
| Applications |
ELISA
IHC-P
IHC-F
IF
|
| Reactivity | Human, Mouse, Rat |
| Specifications | |
| Conjugation | Unconjugated |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human HMBS/PBGD |
| Immunogen Range | 21-120/361 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles. |
| Target | |
| Gene ID | 3145 |
| Synonyms | HEM3_HUMAN; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; PBGD; Porphobilinogen deaminase; Pre uroporphyrinogen synthase; Pre-uroporphyrinogen synthase; UPS; Uroporphyrinogen I synthase; Uroporphyrinogen I synthetase. |
| Background | PBGD, 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. |
| Application Dilution | |
| ELISA | WB1:300-5000 |
| IHC-P | 1:200-400 |
| IHC-F | IFIHC-P1:50-200 |
| IF | |