Abstract
The administration of fructose (F) to patients with hereditary fructose intolerance induces a profound hypoglycemia which is not relieved by glucagon. This lack of response was tentatively attributed to the partial (70 %} inhibition of phosphorylase α by F-1-P (Biochem. J.134,637,1973).
The administration of tagatose (T) to rats produces changes that are similar to those induced by F (accumulation of ketose 1-P, depletion of ATP and Pi), but last longer due to the slower metabolism of T. In controls, the SC administration of glucagon (0.1 mg/kg) provoked a 60 % rise of hepatic glucose and a 3-fold rise of glucose 6-P after 10 min. In rats treated with T (2 g/kg IP) 10 min before glucagon, both increases were completely abolished notwithstanding a normal activation of phosphorylase. This indicates a complete inhibition of phosphorylase a in vivo. The inhibitory effect of T-1--P on purified liver phosphorylase a was, however, not more pronounced than that of F-1-P.
Supported by the Fonds de la Recherche Scientifique Médicale and by the U.S. Public Health Service (grant AM 9235).
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Van Den Berghe, G., Hue, L. & Hers, H. Inhibition in vivo of the hyperglycemic action of glucagon. Study of the pathogeny of hereditary fructose intolerance. Pediatr Res 8, 910 (1974). https://doi.org/10.1203/00006450-197411000-00077
Issue Date:
DOI: https://doi.org/10.1203/00006450-197411000-00077