The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Peptide ELISA: Antibody detection limit dilution 1/16000.
WB: Use at a concentration of 0.03 - 0.1 µg/ml. Detects a band of approximately 150 kDa (predicted molecular weight: 130 kDa).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.
Carbohydrate biosynthesis; gluconeogenesis.
Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia.