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.
Involvement in disease
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.
PCB was immunoprecipitated from HeLa (human epithelial cell line from cervix adenocarcinoma) whole cell extract with 5μg ab229267. Western blot was performed from the immunoprecipitate using ab229267. Anti-rabbit IgG was used as secondary antibody.
Lane 1: HeLa whole cell extract.
Lane 2: Control IgG instead of ab229267 in HeLa whole cell extract.
Lane 3: ab229267 IP in HeLa whole cell extract.
Western blot - Anti-PCB antibody - C-terminal (ab229267)
All lanes : Anti-PCB antibody - C-terminal (ab229267) at 1/1000 dilution
Lane 1 : HEK-293T (human epithelial cell line from embryonic kidney transformed with large T antigen) whole cell extract Lane 2 : A431 (human epidermoid carcinoma cell line) whole cell extract Lane 3 : HeLa (human epithelial cell line from cervix adenocarcinoma) whole cell extract Lane 4 : HepG2 (human liver hepatocellular carcinoma cell line) whole cell extract