[Glycogen disease caused by disorders in the liver phosphorylase system]. 1979

I V Chibisov, and E L Rozenfel'd, and L V Chistova, and A F Leont'ev

Concentration of glycogen and activity of enzymes, participating in its metabolism, were studied in punctates of liver tissue obtained from three patients with clinical manifestations of hepatic forms of glycogenosis. Accumulation of glycogen in liver tissue of the patients was caused by distinct decrease in the phosphorylase activity. In two patients low activity of phosphorylase A was due to impairment of one of the enzymes, activating the phosphorylase in liver tissue, and in one patient phosphorylase B itself exhibited low activity. The in vitro data on glycogen metabolism were correlated with those, obtained in study of glycogen turnover in vivo, after loading with adrenaline. The data obtained enable to develop methods for treatment of the patients with impairments in activity of liver phosphorylase.

UI MeSH Term Description Entries
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008112 Liver Glycogen Glycogen stored in the liver. (Dorland, 28th ed) Hepatic Glycogen,Glycogen, Hepatic,Glycogen, Liver
D008297 Male Males
D010762 Phosphorylase a The active form of GLYCOGEN PHOSPHORYLASE that is derived from the phosphorylation of PHOSPHORYLASE B. Phosphorylase a is deactivated via hydrolysis of phosphoserine by PHOSPHORYLASE PHOSPHATASE to form PHOSPHORYLASE B.
D010763 Phosphorylase b The inactive form of GLYCOGEN PHOSPHORYLASE that is converted to the active form PHOSPHORYLASE A via phosphorylation by PHOSPHORYLASE KINASE and ATP.
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D006008 Glycogen Storage Disease A group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. In some patients, prominent liver involvement is presented. In others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement. Glycogenosis,Disease, Glycogen Storage,Diseases, Glycogen Storage,Glycogen Storage Diseases,Glycogenoses,Storage Disease, Glycogen,Storage Diseases, Glycogen
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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