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.
D000293:Adolescent; D000328:Adult; D000596:Amino Acids / Q000097:blood / Q000302:isolation & purification*; D001786:Blood Glucose / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005934:Glucagon / Q000097:blood; D006008:Glycogen Storage Disease / Q000175:diagnosis*; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000175:diagnosis*; D006010:Glycogen Storage Disease Type III / Q000097:blood / Q000175:diagnosis*; D006801:Humans; D007328:Insulin / Q000097:blood; D007773:Lactates / Q000097:blood; D019344:Lactic Acid; D008297:Male; D008875:Middle Aged
D000596:Amino Acids; D001786:Blood Glucose; D007328:Insulin; D007773:Lactates; D019344:Lactic Acid; D005934:Glucagon