PT - JOURNAL ARTICLE AU - Cheng, Alan AU - Saltiel, Alan R. TI - Von Gierke’s Disease Adopts an Orphan (and Its Partner) AID - 10.1126/scisignal.258pe8 DP - 2009 Feb 17 TA - Science Signaling PG - pe8--pe8 VI - 2 IP - 58 4099 - http://stke.sciencemag.org/content/2/58/pe8.short 4100 - http://stke.sciencemag.org/content/2/58/pe8.full SO - Sci. Signal.2009 Feb 17; 2 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.