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 13C labeled hepatic UDP glucose from administered 1-13C galactose. NMR analysis of urinary acetaminophen glucuronide, which is derived from hepatic UDP glucose showed 13C enrichment after concomitant administration of 13C galactose and acetaminophen. The finding is consistent with the function of UDP galactose pyrophosphorylase as an alternate pathway of galactose metabolism.