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Pyruvate carboxylase, mitochondrial (PC)

UniProt Number: P11498
Alternate Names: Pyruvic carboxylase, PCB
Structure and Function: Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin cofactor in the first step and the transfer of this carboxyl group to pyruvate in the second making oxaloacetate. Therefore, pyruvate carboxylase catalyzes the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis. In addition to being a key regulator of gluconeogenesis, pyruvate carboxylase has an anaplerotic role providing oxaloacetate to the TCA cycle when needed.
Disease Associations: 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.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
MS774 PC antibody ICC, ICE, IP, FLOW human, mouse, rat, bovine 100 µg
ab123451 Anti-PCB antibody [10G7BF7] (ab123451) ICE, WB, ICC Mouse, Rat, Human 100 µg

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Pyruvate Metabolism: Key Nodes


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