ACCÈS RAPIDE AUX LIENS
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PGBS (porphobilinogen synthase), an enzyme that belongs to the ALADH family, is composed of eight identical subunits and catalyzes the condensation of two molecules of δ-aminolevulinate to form porphobilinogen, a precursor of heme, cytochromes and other hemoproteins. It also catalyzes the second step in the porphyrin and heme biosynthetic pathway in which zinc is essential for enzymatic activity. PGBS is inhibited by lead. A defect in the gene encoding PGBS, ALAD, can cause increased sensitivity to lead poisoning and acute hepatic porphyria, a group of inherited disorders caused by partial enzyme defects in heme biosynthesis, which includes acute intermittent porphyria, variegate porphyria and hereditary coproporphyria. There are two common alleles of ALAD, ALAD*2 and ALAD*1. When exposed to environmental lead, individuals heterozygous or homozygous for ALAD*2 Asn 59 have significantly higher blood lead levels than do ALAD*1 Lys 59 homozygotes.
Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps PBGS (E-10) | sc-398308 | 200 µg/ml | $316.00 | |||
PBGS (E-10): m-IgG Fc BP-HRP Kit | sc-540929 | 200 µg Ab; 10 µg BP | $354.00 | |||
PBGS (E-10): m-IgG1 BP-HRP Kit | sc-542470 | 200 µg Ab; 20 µg BP | $354.00 | |||
PBGS (E-10) peptide neutralisant | sc-398308 P | 100 µg/0.5 ml | $68.00 |