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Galactose-1-phosphate uridyltransferase (or GALT, G1PUT) is an enzyme (EC 2.7.7.12) responsible for converting ingested galactose to glucose. [ 5 ] Galactose-1-phosphate uridyltransferase (GALT) catalyzes the second step of the Leloir pathway of galactose metabolism, namely:
In classic galactosemia, galactose-1-phosphate uridylyltransferase activity is reduced or absent; leading to an accumulation of the precursors, galactose, galactitol, and Gal-1-P. [3] The elevation of precursors can be used to differentiate GALT deficiency from galactokinase deficiency, as Gal-1-P is typically not elevated in galactokinase ...
D-Galactose-1-phosphate is an intermediate in the intraconversion of glucose and uridine diphosphate galactose. [1] It is formed from galactose by galactokinase.The improper metabolism of galactose-1-phosphate is a characteristic of galactosemia. [2] The Leloir pathway is responsible for such metabolism of galactose and its intermediate ...
In galactose metabolism, the enzyme galactose 1-phosphate uridylyltransferase transfers a phosphate from UDP-glucose to galactose 1-phosphate to produce UDP-galactose, which is then converted to UDP-glucose. [15] Bacteria with defective UTP—glucose-1-phosphate uridylyltransferase are unable to incorporate galactose into their cell walls. [16]
Next, α-D-galactose is phosphorylated by galactokinase to galactose 1-phosphate. In the third step, D-galactose-1-phosphate uridylyltransferase converts galactose 1-phosphate to UDP-galactose using UDP-glucose as the uridine diphosphate source. Finally, UDP-galactose 4-epimerase recycles the UDP-galactose to UDP-glucose for the transferase ...
Unlike galactose-1-phosphate uridyltransferase deficiency, the symptoms of galactokinase deficiency are relatively mild. The only known symptom in affected children is the formation of cataracts, due to the production of galactitol in the lens of the eye. [6]
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The systematic name of this enzyme class is UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase. Other names in common use include uridyl transferase, hexose-1-phosphate uridylyltransferase, uridyltransferase, and hexose 1-phosphate uridyltransferase. This enzyme participates in galactose metabolism and nucleotide sugars metabolism.