HMBS Polyclonal Antibody-BS90649

HMBS Polyclonal Antibody

Sizes: 50µl, 100µl

Catalogue Numbers: BS90649-50, BS90649-100

Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Swiss-Prot: P08397(Human) P22907(Mouse) P19356(Rat)

Host: Rabbit

Reactivity: Human, Mouse, Rat

Applications: WB

All Applications: WB:1:1,000-1:2,000

Background: 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.

Purification and Purity: ProA affinity purified

Storage and Stability: Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: Western blot analysis of HMBS on differentLysates using anti-HMBS antibody at 1/1,000 dilution. Positive control: Lane 1: Hela Lane 2: 293T

Bioworld Molecular Weight: 40 kDa

Specificity: HMBS polyclonal antibody detects endogenous levels of HMBS protein.

Alternative Name: PBG-D, HMBS, HEM3, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, PORC, Porphobilinogen deaminase, porphyria, acute; Chester type, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase,

Immunogen: recombinant protein

Conjugate: Unconjugated

Modification: Unmodified