| Overview |
| bs-9068R |
| HMBS Polyclonal Antibody |
| ELISA, IHC-P, IHC-F, IF |
| Human, Mouse, Rat |
| Specifications |
| Unconjugated |
| Rabbit |
| KLH conjugated synthetic peptide derived from human HMBS/PBGD |
| Polyclonal |
| #REF! |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles. |
| Target |
| 3145 |
| 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. |
| 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 |
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