{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:24:26Z","timestamp":1788305066340,"version":"build-2803163510"},"reference-count":31,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2010,2,2]],"date-time":"2010-02-02T00:00:00Z","timestamp":1265068800000},"content-version":"vor","delay-in-days":1,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2010,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>A potential role for muscle in glucose homeostasis was recently suggested based on characterization of extrahepatic and extrarenal glucose\u20106\u2010phosphatase (glucose\u20106\u2010phosphatase\u2010\u03b2). To study the role of extrahepatic tissue in glucose homeostasis during fasting glucose kinetics were studied in two patients with a deficient hepatic and renal glycogenolysis and\/or gluconeogenesis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design<\/jats:title><jats:p>Endogenous glucose production (EGP), glycogenolysis (GGL), and gluconeogenesis (GNG) were quantified with stable isotopes in a patient with glycogen storage disease type 1a (GSD\u20101a) and a patient with fructose\u20101,6\u2010bisphosphatase (FBPase) deficiency. The [6,6\u2010<jats:sup>2<\/jats:sup>H<jats:sub>2<\/jats:sub>]glucose dilution method in combination with the deuterated water method was used during individualized fasting tests.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Both patients became hypoglycemic after 2.5 and 14.5 h fasting, respectively. At that time, the patient with GSD\u20101a had EGP 3.84 \u03bcmol\/kg per min (30% of normal EGP after an overnight fast), GGL 3.09 \u03bcmol\/kg per min, and GNG 0.75 \u03bcmol\/kg per min. The patient with FBPase deficiency had EGP 8.53 \u03bcmol\/kg per min (62% of normal EGP after an overnight fast), GGL 6.89 \u03bcmol\/kg per min GGL, and GNG 1.64 \u03bcmol\/kg per min.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>EGP was severely hampered in both patients, resulting in hypoglycemia. However, despite defective hepatic and renal GNG in both disorders and defective hepatic GGL in GSD\u20101a, both patients were still able to produce glucose via both pathways. As all necessary enzymes of these pathways have now been functionally detected in muscle, a contribution of muscle to EGP during fasting via both GGL as well as GNG is suggested.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s10545-009-9030-9","type":"journal-article","created":{"date-parts":[[2010,2,1]],"date-time":"2010-02-01T07:57:40Z","timestamp":1265011060000},"page":"25-31","source":"Crossref","is-referenced-by-count":21,"title":["A potential role for muscle in glucose homeostasis: in vivo kinetic studies in glycogen storage disease type 1a and fructose\u20101,6\u2010bisphosphatase deficiency"],"prefix":"10.1002","volume":"33","author":[{"given":"Hidde H.","family":"Huidekoper","sequence":"first","affiliation":[{"name":"Department of Pediatrics (G8\u2010205) Academic Medical Center University Hospital of Amsterdam PO Box 22660 Amsterdam NL\u20101100 DD The 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