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.

Specificity:

HMBS polyclonal antibody detects endogenous levels of HMBS protein.

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,

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Ig type:

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

Immunogen:

recombinant protein

Purification & Purity:

ProA affinity purified

Molecular weight:

40 kDa

Applications:

WB

Usage:

WB:1:1,000-1:2,000

Storage:

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

Precautions:

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

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