Galactokinase catalyzes the first step of galactose phosphorylation in the Leloir pathway of intermediate metabolism. Galactokinase deficiency is one of the three inborn errors of metabolism that lead to hypergalactosemia.
Galactokinase initiates the first step in the Leloir pathway for galactose phosphorylation, a critical part of intermediary metabolism. A deficiency in this enzyme can result in hypergalactosemia, one of three primary congenital metabolic disorders.