[Regulation of glycogen metabolism in the liver and hepatic glycogenosis due to phosphorylase system deficiency].
Glycogen synthesis and breakdown in the liver are tightly controlled through different mechanisms. The purpose of this review is to describe some properties of the enzymes involved in the glycogen metabolism and the sequence of events by which glucose, allosteric effectors and hormones control this metabolism in the liver. Clinical, genetic and biological aspects of the phosphorylase and the phosphorylase kinase deficiencies are examined. The enzymatic analysis of the haemolysates from the patients allows discrimination of these two types of glycogenosis.
D006008:Glycogen Storage Disease / Q000201:enzymology*; D006006:Glycogen Synthase / Q000378:metabolism; D008112:Liver Glycogen / Q000096:biosynthesis / Q000378:metabolism*; D010749:Phosphoprotein Phosphatases / Q000378:metabolism; D010744:Phosphoric Monoester Hydrolases / Q000378:metabolism; D010764:Phosphorylase Kinase / Q000378:metabolism; D010762:Phosphorylase a / Q000502:physiology; D006005:Phosphorylases / Q000172:deficiency*; D010770:Phosphotransferases / Q000172:deficiency*; D011494:Protein Kinases / Q000378:metabolism
D008112:Liver Glycogen; D010762:Phosphorylase a; D006005:Phosphorylases; D006006:Glycogen Synthase; D010770:Phosphotransferases; D011494:Protein Kinases; D010764:Phosphorylase Kinase; D010749:Phosphoprotein Phosphatases; D010744:Phosphoric Monoester Hydrolases
La régulation du métabolisme du glycogène dans le foie et les glycogénoses hépatiques par déficit du système phosphorylasique.