Evidence for function of UDP galactose pyrophosphorylase in mice with absent galactose-1-phosphate uridyltransferase.
Mice with deletion of the galactose-1-phosphate uridyltransferase (GALT) gene were examined for their ability to form (13)C labeled hepatic UDP glucose from administered 1-(13)C galactose. NMR analysis of urinary acetaminophen glucuronide, which is derived from hepatic UDP glucose showed (13)C enrichment after concomitant administration of (13)C galactose and acetaminophen. The finding is consistent with the function of UDP galactose pyrophosphorylase as an alternate pathway of galactose metabolism.
Wehrli
Suzanne
S
NMR Core Facility, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Reynolds
Robert
R
Segal
Stanton
S
D000818:Animals; D002247:Carbon Isotopes; D005690:Galactose / Q000378:metabolism; D008099:Liver / Q000201:enzymology / Q000378:metabolism; D009682:Magnetic Resonance Spectroscopy; D051379:Mice; D018345:Mice, Knockout; D005694:UTP-Hexose-1-Phosphate Uridylyltransferase / Q000172:deficiency / Q000235:genetics / Q000502:physiology*; D014532:Uridine Diphosphate Glucose / Q000378:metabolism
D002247:Carbon Isotopes; D005694:UTP-Hexose-1-Phosphate Uridylyltransferase; D014532:Uridine Diphosphate Glucose; D005690:Galactose