TY - JOUR
T1 - Amino acid disturbances in type III glycogenosis
T2 - Differences from type I glycogenosis
AU - Slonim, Alfred E.
AU - Coleman, Rosalind A.
AU - Moses, Shimon
AU - Bashan, Nava
AU - Shipp, Elizabeth
AU - Mushlin, Philip
N1 - Funding Information:
From The Departments of Pediatrics. Vanderbilt University Medical Center Nashville. Tennessee, Duke University Medical Center Durham, North Carolina, Soroka Medical Center Faculty of Health Sciences, Ben-Gurion University of the Negev Beersheba, Israel. Receivedforpublication Jury 13. 1982. Address reprint requests to Alfred E. Slonim. M.D.. Dept. of Pediatrics, Vanderbilt University, Medical Center North, Nashville. Tennessee 37232. Supported in part by clinical research grant 6-239 from the National Foundation-March of Dimes, Clinical Research Center Grants SMOI RR-95 and RR-30 from NIH, Vanderbilt University Research Council grant, and BRSG RR 05424-21. 0 1983 by Grune & Stratton, Inc. 0026~495/83/32014l014$01.00/0
PY - 1983/1/1
Y1 - 1983/1/1
N2 - 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 storagen storage disease (GSD-I) patients. The oustanding 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 possiblity 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.
AB - 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 storagen storage disease (GSD-I) patients. The oustanding 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 possiblity 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.
UR - http://www.scopus.com/inward/record.url?scp=0020664099&partnerID=8YFLogxK
U2 - 10.1016/0026-0495(83)90159-2
DO - 10.1016/0026-0495(83)90159-2
M3 - Article
AN - SCOPUS:0020664099
SN - 0026-0495
VL - 32
SP - 70
EP - 74
JO - Metabolism: Clinical and Experimental
JF - Metabolism: Clinical and Experimental
IS - 1
ER -