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Pyruvate Carboxylase requires biotin and ATP to catalyse the carboxylation of pyruvate to oxaloacetate. The active enzyme is a homotetramer arranged in a tetrahedron which is located exclusively in the mitochondrial matrix. Pyruvate carboxylase is involved in gluconeogenesis, lipogenesis, insulin secretion and synthesis of the neurotransmitter glutamate. Mutations in this gene have been associated with pyruvate carboxylase deficiency.
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Protein Aliases: EC 220.127.116.11; PCB; PYC; Pyruvate carboxylase, mitochondrial; pyruvate decarboxylase; Pyruvic carboxylase
Gene Aliases: PC; PCB; Pcx