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  • Title: Liver glycogenosis caused by a defective phosphorylase system: hemolysate analysis.
    Author: Baussan C, Moatti N, Odievre M, Lemonnier A.
    Journal: Pediatrics; 1981 Jan; 67(1):107-12. PubMed ID: 6787554.
    Abstract:
    Investigated were 24 cases of glycogenosis caused by a reduction in liver phosphorylase activity. The intravenous glucagon tolerance test could not discriminate between phosphorylase kinase deficiency [glycogen storage disease (GSD) IX] and phosphorylase deficiency (GSD VI). These two subgroups were distinguished by hemolysate enzyme assays: (1) GSD IX was characterized by a residual phosphorylase kinase activity, a low activation curve for endogenous phosphorylase b and increased amylo-1,6-glucosidase activity. (2) GSD VI was characterized by a normal or increased phosphorylase kinase activity, a slight activation of endogenous phosphorylase b and a normal amylo-1,6-glucosidase activity.
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