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.