PCB polyclonal antibody

Pyruvate carboxylase (PCB) is a biotinylated mitchondrial enzyme that catalyzes the synthesis of oxaloacetate from pyruvate in a tissue specific manner. In addition to covalently binding the biotin cofactor, PCB contains consensus sequences for the attachment of ATP and the substrate pyruvate. The PCB gene is located on the long arm of chromosome 11. Mutations in PCB metabolism (pyruvate carboxylase deficiency) are known to cause lactic acidosis, hypoglycemia and mental retardation.