[HTML][HTML] A glucose-6-phosphate hydrolase, widely expressed outside the liver, can explain age-dependent resolution of hypoglycemia in glycogen storage disease …

JJ Shieh, CJ Pan, BC Mansfield, JY Chou - Journal of Biological Chemistry, 2003 - ASBMB
A fine control of the blood glucose level is essential to avoid hyper-or hypo-glycemic shocks
associated with many metabolic disorders, including diabetes mellitus and type I glycogen
storage disease. Between meals, the primary source of blood glucose is gluconeogenesis
and glycogenolysis. In the final step of both pathways, glucose-6-phosphate (G6P) is
hydrolyzed to glucose by the glucose-6-phosphatase (G6Pase) complex. Because G6Pase
(renamed G6Pase-α) is primarily expressed only in the liver, kidney, and intestine, it has …