Article
Pathways of galactose metabolism by galactosemics: evidence for galactose conversion to hepatic UDPglucose.
Molecular genetics and metabolism - 1 Feb 2006
Segal Stanton, Wehrli Suzanne, Yager Claire, Reynolds Robert
Abstract excerpt
To determine if classic galactosemics have residual galactose-1-phosphate uridyltransferase (GALT) activity to explain their considerable ability to oxidize galactose over 24 h, we devised a method for assessing their ability to form hepatic UDPglucose (UDPglu), an intermediate in the normal Leloir pathway of galactose metabolism. The protocol involved the single oral administration of 7 mg/kg [2-13C]galactose...
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