galaktokinase

Galaktokinase is an enzyme that catalyzes the phosphorylation of galactose. Specifically, it converts galactose to galactose-1-phosphate using adenosine triphosphate (ATP) as the phosphate donor. This reaction is the first step in the Leloir pathway, the primary route for galactose metabolism in humans and many other organisms. The enzyme is also known as GALK.

The importance of galaktokinase lies in its role in processing dietary galactose, a sugar commonly found in milk products. Without functional galaktokinase, galactose cannot be effectively metabolized, leading to its accumulation in the body. This accumulation can have significant health consequences, particularly for infants, as it can interfere with the metabolism of other sugars and contribute to the development of cataracts.

Galaktokinase is found in various tissues, including the liver, kidneys, and lens of the eye. Its activity is crucial for providing the necessary intermediate for further metabolic steps, ultimately leading to the production of glucose-1-phosphate, which can then enter glycolysis for energy production. Genetic deficiencies in galaktokinase are rare but can lead to galactosemia, a condition characterized by elevated galactose levels.