Amino acid disturbances in type III glycogenosis: differences from type I glycogenosis.

The plasma glucose, insulin, glucagon, lactate and amino acid response patterns to glucose and protein meals were examined in 11 patients with type III glycogen storage disease (GSD-III). The amino acid metabolism in GSD-III was shown to differ from that observed in normal subjects and in type I glycogen storage disease (GSD-I) patients. The outstanding findings involved the principal gluconeogenic amino acid, alanine. Postabsorptive levels of alanine in GSD-III were significantly below those of normal controls. Following glucose ingestion, alanine rose markedly in GSD-III, which differed from normal subjects in whom no change occurred, and from GSD-I patients in whom a sharp fall was observed. Following beef ingestion, the direction of change of alanine was similar in the three groups, but the circulating levels in GSD-III were significantly less than those observed in GSD-I and normal controls. The possibility that gluconeogenesis is enhanced in GSD-III was supported by the prompt rise in blood glucose observed following beef ingestion, which differed from GSD-I and normal subjects, in which no rise in glucose was observed.