It is a key regulatory enzyme in. Pyruvate carboxylase deficiency Other Names. Gluconeogenesis Why This Is Very Important Simple Notes Biochemistry Notes Teaching Biology Biology Notes Leigh syndrome due to pyruvate. . Pyruvate carboxylase deficiency occurs when an enzyme called pyruvate carboxylase does not work correctly. The active enzyme is a homotetramer arranged in a tetrahedron which is located. It is present in many. Pyruvate carboxylase PC is a mitochondrial enzyme that catalyzes the ATP-dependent carboxylation of pyruvate to oxaloacetate OAA serving to replenish the. This gene encodes pyruvate carboxylase which requires biotin and ATP to catalyse the carboxylation of pyruvate to oxaloacetate. It is present in many. Pyruvate carboxylase deficiency Description Pyruvate carboxylase deficiency is an inherited disorder that causes lactic acid and other potentially toxic compounds to accumulate in the.