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[Biological and physiopathological aspects of hepatic glycogenoses]
C Baussan1, N Moatti, A Lemonnier
1Laboratoire central de Biochimie, Hôpital Bicêtre, Le Kremlin-Bicêtre.
Annales De Gastroenterologie Et D'Hepatologie
|March 1, 1988
Abstract:
Liver glycogenosis (GSD) are hereditary diseases caused by deficiencies of the three major enzymatic systems involved in glycogenolysis: glucose-6-phosphatase (GSD I), debranching enzyme (GSD III) and phosphorylase system (GSD VI). Biological and physiopathological aspects of these disorders are described. An up to date diagnostic process which includes measurement of glycogen and enzymatic activities, in the most appropriate tissue material, is proposed.