HMBS Recombinant Rabbit monoclonal Antibody IgG
Fig1: Western blot analysis of HMBS on different lysates using anti-HMBS antibody at 1/1,000 dilution.
Lane 1: Hela
Lane 2: 293T
Host Species; Species ReactivityRabbit; Human, Mouse, Rat
Purification; FormulationProA affinity purified; 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.; Liquid form.
ALTnamesPorphobilinogen deaminase, Hydroxymethylbilane synthase, Pre-uroporphyrinogen synthase
BackgroundPBGD (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.(ET1610-91)