Pyruvate Carboxylase (PC) (丙酮酸羧化酶)

Pyruvate carboxylase (PC) is a biotin-dependent mitochondrial enzyme that catalyzes the conversion of pyruvate to oxaloacetate, a critical step in the tricarboxylic acid (TCA) cycle. It typically functions as a tetramer composed of four identical subunits, each with a molecular weight of approximately 120-130 kDa. PC is essential for various anabolic processes, including gluconeogenesis, lipogenesis, insulin secretion, and the synthesis of glutamate neurotransmitters^[1].

In cancer cells, PC plays a pivotal role in metabolic reprogramming and adaptation, contributing to lipid metabolism and protection against oxidative stress. Clinically, pyruvate carboxylase deficiency is a rare metabolic disorder characterized by lactic acidemia, encephalopathy, and prominent neurological symptoms during the neonatal and infantile periods. This highlights PC's critical role in energy metabolism and brain function^[2].