RT Journal Article SR Electronic T1 Von Gierke’s Disease Adopts an Orphan (and Its Partner) JF Science Signaling JO Sci. Signal. FD American Association for the Advancement of Science SP pe8 OP pe8 DO 10.1126/scisignal.258pe8 VO 2 IS 58 A1 Cheng, Alan A1 Saltiel, Alan R. YR 2009 UL http://stke.sciencemag.org/content/2/58/pe8.abstract AB The enzyme glucose-6-phosphatase is critical for maintaining fasting blood sugar levels by increasing hepatic glucose production. Its absence in patients with von Gierke’s disease leads to severe hypoglycemia and abnormal accumulation of glycogen (glycogenosis) in the liver. New players that control the expression of glucose-6-phosphatase have been identified that may provide insight into this metabolic disorder, as well as type 2 diabetes.