Invitrogen™ HMBS Polyclonal Antibody

Descrizione

Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 μg/mL. Positive Control - WB: human Hela whole cell, human placenta tissue, human HepG2 whole cell, human 22RV1 whole cell, human U20S whole cell.

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.