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.