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The main pathway of galactose metabolism is the Leloir pathway; humans and other species, however, have been noted to contain several alternate pathways, such as the De Ley Doudoroff Pathway. The Leloir pathway consists of the latter stage of a two-part process that converts β-D-galactose to UDP-glucose. The initial stage is the conversion of ...
Intermediates and enzymes in the Leloir pathway of galactose metabolism [5] In the first step, galactose mutarotase facilitates the conversion of β-D-galactose to α-D-galactose since this is the active form in the pathway. Next, α-D-galactose is phosphorylated by galactokinase to galactose 1-phosphate.
Galactokinase is an enzyme (phosphotransferase) that facilitates the phosphorylation of α-D-galactose to galactose 1-phosphate at the expense of one molecule of ATP. [1] Galactokinase catalyzes the second step of the Leloir pathway, a metabolic pathway found in most organisms for the catabolism of α-D-galactose to glucose 1-phosphate. [2]
Causes cataracts, which form due to the elevation of galactitol that accumulates when galactose is metabolized in an alternative pathway that is not the Leloir pathway. [2] These are treatable by restricting galactose from the diet. UDPgalactose-4-epimerase deficiency: UDPgalactose-4-epimerase: Is extremely rare (only 2 reported cases).
Carbohydrate metabolism is the whole of the biochemical processes responsible for the metabolic formation, breakdown, and interconversion of carbohydrates in living organisms. Carbohydrates are central to many essential metabolic pathways . [ 1 ]
The enzyme UDP-glucose 4-epimerase (EC 5.1.3.2), also known as UDP-galactose 4-epimerase or GALE, is a homodimeric epimerase found in bacterial, fungal, plant, and mammalian cells. This enzyme performs the final step in the Leloir pathway of galactose metabolism, catalyzing the reversible conversion of UDP-galactose to UDP-glucose. [1]
Additionally, since the metabolism of galactose in the cell is involved in both anabolic and catabolic pathways, a novel regulatory system using two promoters for differential repression has been identified and characterized within the context of the gal operon.
GALT catalyzes the second reaction of the Leloir pathway of galactose metabolism through ping pong bi-bi kinetics with a double displacement mechanism. [6] This means that the net reaction consists of two reactants and two products (see the reaction above) and it proceeds by the following mechanism: the enzyme reacts with one substrate to generate one product and a modified enzyme, which goes ...