{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T18:21:12Z","timestamp":1774376472320,"version":"3.50.1"},"reference-count":41,"publisher":"American Physiological Society","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Endocrinology and Metabolism"],"published-print":{"date-parts":[[2002,6,1]]},"abstract":"<jats:p> Insulin action is decreased by high muscle glycogen concentrations in skeletal muscle. Patients with McArdle's disease have chronic high muscle glycogen levels and might therefore be at risk of developing insulin resistance. In this study, six patients with McArdle's disease and six matched control subjects were subjected to an oral glucose tolerance test and a euglycemic-hyperinsulinemic clamp. The muscle glycogen concentration was 103 \u00b1 45% higher in McArdle patients than in controls. Four of six McArdle patients, but none of the controls, had impaired glucose tolerance. The insulin-stimulated glucose utilization and the insulin-stimulated increase in glycogen synthase activity during the clamp were significantly lower in the patients than in controls (51.3 \u00b1 6.0 vs. 72.6 \u00b1 13.1 \u03bcmol \u00b7 min<jats:sup>\u22121<\/jats:sup> \u00b7 kg lean body mass<jats:sup>\u22121<\/jats:sup>, P &lt; 0.05, and 53 \u00b1 15 vs. 79 \u00b1 9%, P &lt; 0.05, n = 6, respectively). The difference in insulin-stimulated glycogen synthase activity between the pairs was significantly correlated ( r = 0.96, P &lt; 0.002) with the difference in muscle glycogen level. The insulin-stimulated increase in Akt phosphorylation was smaller in the McArdle patients than in controls (45 \u00b1 13 vs. 76 \u00b1 13%, P &lt; 0.05, respectively), whereas basal and insulin-stimulated glycogen synthase kinase 3\u03b1 and protein phosphatase-1 activities were similar in the two groups. Furthermore, the ability of insulin to decrease and increase fat and carbohydrate oxidation, respectively, was blunted in the patients. In conclusion, these data show that patients with McArdle's glycogen storage disease are insulin resistant in terms of glucose uptake, glycogen synthase activation, and alterations in fuel oxidation. The data further suggest that skeletal muscle glycogen levels play an important role in the regulation of insulin-stimulated glycogen synthase activity. <\/jats:p>","DOI":"10.1152\/ajpendo.00526.2001","type":"journal-article","created":{"date-parts":[[2015,2,27]],"date-time":"2015-02-27T20:09:54Z","timestamp":1425067794000},"page":"E1267-E1275","source":"Crossref","is-referenced-by-count":29,"title":["Decreased insulin action in skeletal muscle  from patients with McArdle's disease"],"prefix":"10.1152","volume":"282","author":[{"given":"Jakob N.","family":"Nielsen","sequence":"first","affiliation":[{"name":"Copenhagen Muscle Research Center, Department of Human Physiology, Institute of Exercise and Sport Sciences, University of Copenhagen, DK-2400 Copenhagen;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Vissing","sequence":"additional","affiliation":[{"name":"Copenhagen Muscle Research Center, Department of Neurology, Rigshospitalet, DK-2100 Copenhagen, Denmark;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00f8rgen F. 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