Galactokinase is an enzyme encoded by the GALK1 gene. It is chiefly responsible for catalyzing the first step in the Leloir pathway of galactose metabolism, which converts galactose into galactose-1-phosphate. This enzyme is predominantly found in the liver, where carbohydrate metabolism primarily occurs. The activity of galactokinase is essential for maintaining normal levels of galactose in the blood, preventing its accumulation and potential toxic effects.