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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.
Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps PBGD (B-6) | sc-166788 | 200 µg/ml | $316.00 | |||
PBGD (B-6): m-IgG Fc BP-HRP Kit | sc-537531 | 200 µg Ab; 10 µg BP | $354.00 | |||
PBGD (B-6): m-IgGκ BP-HRP Kit | sc-534824 | 200 µg Ab; 40 µg BP | $354.00 | |||
PBGD (B-6): m-IgG1 BP-HRP Kit | sc-545215 | 200 µg Ab; 20 µg BP | $354.00 | |||
PBGD (B-6) peptide neutralisant | sc-166788 P | 100 µg/0.5 ml | $68.00 |