ACCÈS RAPIDE AUX LIENS
VOIR ÉGALEMENT...
Uroporphyrinogen decarboxylase, also known as UROD or UPD, is a 367 amino acid protein that exists as a homodimer. UROD is the fifth enzyme in the human heme biosynthetic pathway and is responsible for the conversion of uroporphyrinogen to coproporphyrinogen through the removal of four carboxymethyl side chains. Mutations in the UROD gene are responsible for three autosomal disorders in humans: familial porphyria cutanea tarda (f-PCT), sporadic porphyria cutanea tarda (s-PCT) and hepatoerythropoietic porphyria (HEP). F-PCT is an autosomal dominant disorder characterized by late-onset light-sensitive dermititis. High levels of uroporphyrin excretion in the urine and varying degrees of liver damage are associated with this disease. S-PCT is an idiosyncratic form of PCT that is characterized by a reduction of liver enzymes. HEP is an autosomal recessive disorder that affects infants. It is characterized by excessive excretion of acetate-substituted porphyrins and accumulation of protoporphyrin in erythrocytes.
Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps UROD (G-7) | sc-374318 | 200 µg/ml | $316.00 | |||
UROD (G-7): m-IgG Fc BP-HRP Kit | sc-540434 | 200 µg Ab; 10 µg BP | $354.00 | |||
UROD (G-7): m-IgG1 BP-HRP Kit | sc-542120 | 200 µg Ab; 20 µg BP | $354.00 |