Evidence for function of UDP galactose pyrophosphorylase in mice with absent galactose-1-phosphate uridyltransferase

Mol Genet Metab. 2007 Jun;91(2):191-4. doi: 10.1016/j.ymgme.2007.02.013. Epub 2007 Apr 11.

Abstract

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.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Carbon Isotopes
  • Galactose / metabolism
  • Liver / enzymology
  • Liver / metabolism
  • Magnetic Resonance Spectroscopy
  • Mice
  • Mice, Knockout
  • UTP-Hexose-1-Phosphate Uridylyltransferase / deficiency
  • UTP-Hexose-1-Phosphate Uridylyltransferase / genetics
  • UTP-Hexose-1-Phosphate Uridylyltransferase / physiology*
  • Uridine Diphosphate Glucose / metabolism

Substances

  • Carbon Isotopes
  • UTP-Hexose-1-Phosphate Uridylyltransferase
  • Uridine Diphosphate Glucose
  • Galactose