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Leloir Pathway for Galactose Metabolism

This image is a scaled-down version of the actual pathway image. It does not contain any links to the protein information pages.


In most organisms, the conversion of Galactose to the more metabolically useful Glucose-1-Phosphate is accomplished by the action of four enzymes that constitute the Leloir pathway. In the first step of this pathway, Beta-D-Galactose is epimerized to Alpha-D-Galactose by GALM (Galactose Mutarotase/Aldose 1-Epimerase) (Ref.1). The active site of GALM is positioned in a rather open cleft with the hydroxyl groups of Galactose lying within hydrogen bonding distance to a number of side chains, the reaction catalyzed by human GALM proceeds via Glu-307 (Glutamic acid-307) and His-176 (Histidine-176). Till now no diseases have been attributed to mutations in human GALM. The next step involves the ATP-dependent phosphorylation of Alpha-D-Galactose by GalK (Galactokinase) to yield Galactose-1-Phosphate. Deficiencies in this enzyme lead to [...]


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