Pathways
Leloir Pathway for Galactose Metabolism
Description:
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 ...
References:
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Structure and function of enzymes of the Leloir pathway for galactose metabolism.
J. Biol. Chem. 2003 Nov 7;278(45):43885-8. -
The Leloir pathway: a mechanistic imperative for three enzymes to change the stereochemical configuration of a single carbon in galactose.
FASEB J. 1996 Mar;10(4):461-70. -
Structural and kinetic studies of sugar binding to galactose mutarotase from Lactococcus lactis.
J. Biol. Chem. 2002 Nov 22;277(47):45458-65. -
Subcellular localization of galactose-1-phosphate uridylyltransferase in the yeast Saccharomyces cerevisiae.
Mol. Genet. Metab. 2000 Aug;70(4):272-80.
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