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PCCA (propionyl Coenzyme A carboxylase, alpha polypeptide) is a 703 amino acid protein that localizes to the mitochondrion matrix. PCCA is a mitochondrial, biotin-dependent enzyme involved in the catabolism of branched chain amino acids, odd chain fatty acids, and other metabolites. PCCA is responsible for the formation of carboxybiotin upon hydrolysis of ATP and contains a C-terminal biotin-binding domain and a biotin carboxylase domain. Inherited mutations in the gene that encodes PCCA result in a deficiency of the protein, which leads to propionic acidemia (PA), an autosomal recessive inborn error in the catabolism of methionine, isoleucine, threonine, and valine, odd-numbered chain length fatty acids and cholesterol. Two isoforms exists due to alternative splicing events.
Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps PCCA (H-1) | sc-393527 | 200 µg/ml | $316.00 | |||
PCCA (H-1): m-IgG Fc BP-HRP Kit | sc-538238 | 200 µg Ab; 10 µg BP | $354.00 | |||
PCCA (H-1): m-IgGκ BP-HRP Kit | sc-535812 | 200 µg Ab; 40 µg BP | $354.00 | |||
PCCA (H-1): m-IgG1 BP-HRP Kit | sc-545594 | 200 µg Ab; 20 µg BP | $354.00 |