Glycogen metabolism in the liver of the neonatal gsd/gsd and control (GSD/GSD) rat

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Abstract
The metabolism of hepatic glycogen, labeled with [6-3H]glucose at day 19.5 of gestation and with 14C from [U-14C]galactose at delivery, was followed for 10 h in food-deprived gsd/gsd [glycogen storage disease] and control (GSD/GSD) neonatal rats. In the affected pups glycogen was maintained at 12% (wt/wt) and there was no loss of incorporated radioactivity. The 3H and 14C in glycogen from the controls were both decreased by 80%, but 14C was removed at 0-5 h and [6-3H]glucose at 5-10 h. Blood glucose concentrations in the unaffected neonatal rats fell from 5.3 mM at 20 min to 1.7 mM after 10 h. In the gsd/gsd pups blood glucose concentration was decreased from 2 mM at birth to 0.3 mM at 2.5 h; it was maintained at 0.8 mM between 5 and 10 h. In neonatal rats that had been dead for 10 h, hepatic glycogen was decreased by 34% in the controls and by 22% in the gsd/gsd pups. These results demonstrate that liver from the affected rats contains glycogenolytic activity, but that it is not expressed in living tissue.