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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: EC 126.96.36.199; mitochondrial; PCB; PYC; Pyruvate carboxylase, mitochondrial; Pyruvic carboxylase
Gene Aliases: PC; PCB; Pcx
Molecular Function: ligase