Glycogen metabolism in the liver of the neonatal gsd/gsd and control (GSD/GSD) rat.
1. The metabolism of hepatic glycogen, labelled 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 and control (GSD/GSD) neonatal rats. 2. In the affected pups glycogen was maintained at 12% (w/w) and there was no loss of incorporated radioactivity. 3. 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. 4. 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. 5. 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.
D000818:Animals; D000831:Animals, Newborn / Q000097:blood / Q000378:metabolism*; D001786:Blood Glucose / Q000378:metabolism; D006008:Glycogen Storage Disease / Q000097:blood / Q000378:metabolism*; D007773:Lactates / Q000097:blood; D019344:Lactic Acid; D008112:Liver Glycogen / Q000378:metabolism*; D051381:Rats; D011919:Rats, Inbred Strains; D013217:Starvation / Q000097:blood / Q000378:metabolism
D001786:Blood Glucose; D007773:Lactates; D008112:Liver Glycogen; D019344:Lactic Acid