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Combined<jats:sup>1<\/jats:sup>H\/<jats:sup>31<\/jats:sup>P nuclear magnetic resonance spectroscopy (NMRS) was used to assess brain metabolism. Before and after administration of 1 mg glucagon, endogenous glucose production (EGP) was measured with d-[6,6-<jats:sup>2<\/jats:sup>H<jats:sub>2<\/jats:sub>]glucose and hepatic glucose metabolism was examined by<jats:sup>1<\/jats:sup>H\/<jats:sup>13<\/jats:sup>C\/<jats:sup>31<\/jats:sup>P NMRS. At baseline, GSD1 patients exhibited significantly lower rates of EGP (0.53 \u00b1 0.04 vs. 1.74 \u00b1 0.03 mg\u00b7kg<jats:sup>\u22121<\/jats:sup>\u00b7min<jats:sup>\u22121<\/jats:sup>; P &lt; 0.01) but an increased intrahepatic glycogen (502 \u00b1 89 vs. 236 \u00b1 11 mmol\/l; P = 0.05) and lipid content (16.3 \u00b1 1.1 vs. 1.4 \u00b1 0.4%; P &lt; 0.001). After glucagon challenge, EGP did not change in GSD1 patients (0.53 \u00b1 0.04 vs. 0.59 \u00b1 0.24 mg\u00b7kg<jats:sup>\u22121<\/jats:sup>\u00b7min<jats:sup>\u22121<\/jats:sup>; P = not significant) but increased in healthy controls (1.74 \u00b1 0.03 vs. 3.95 \u00b1 1.34; P &lt; 0.0001). In GSD1 patients, we found an exaggerated increase of intrahepatic phosphomonoesters (0.23 \u00b1 0.08 vs. 0.86 \u00b1 0.19 arbitrary units; P &lt; 0.001), whereas inorganic phosphate decreased (0.36 \u00b1 0.08 vs. \u22120.43 \u00b1 0.17 arbitrary units; P &lt; 0.01). Intracerebral ratios of glucose and lactate to creatine were higher in GSD1 patients ( P &lt; 0.05 vs. control). Therefore, hepatic defects of glucose metabolism persist in young adult GSD1 patients. Upregulation of the glucose and lactate transport at the blood-brain barrier could be responsible for the amelioration of hypoglycemic symptoms.<\/jats:p>","DOI":"10.1152\/ajpendo.00658.2006","type":"journal-article","created":{"date-parts":[[2007,9,5]],"date-time":"2007-09-05T00:54:09Z","timestamp":1188953649000},"page":"E1378-E1384","source":"Crossref","is-referenced-by-count":13,"title":["Characterization of hepatic and brain metabolism in young adults with glycogen storage disease type 1: a magnetic resonance spectroscopy study"],"prefix":"10.1152","volume":"293","author":[{"given":"D.","family":"Weghuber","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Mandl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Kr\u0161\u0161\u00e1k","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Roden","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Nowotny","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Brehm","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Krebs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Widhalm","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. 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