HMBS polyclonal Antibody | BS90649

HMBS polyclonal Antibody | BS90649 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human, Mouse, Rat

Application: WB

Application Range: 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.

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

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

Molecular Weight: 40 kDa

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

Alternative Names: 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: Unmodification

Purification & Purity: ProA affinity purified

Pathway: