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Introduction: Glucose-6 phosphatase (Glc6Pase) is an enzyme essential for the regulation of blood glucose homeostasis. It catalyzes the hydrolysis of glucose-6 phosphate (Glc6P) to glucose and inorganic phosphate (Pi). This is the last biochemical reaction common to gluconeogenesis and glycogenolysis. It has been suggested that numerous tissues contain low activities of Glc6Pase (see 1, 2 for reviews). However, it is expressed in significant amounts essentially in the liver and the kidney cortex (1–3), and confers on these two gluconeogenic tissues the capacity to release glucose into the blood (Fig. 1). Inherited deficiency in Glc6Pase (glycogen storage disease (GSD) type 1) is characterized by severe hypoglycemia in the postabsorptive state and patients are prone to long-term troubles such as growth retardation, hepatic steatosis and cirrhosis or renal failure. On the other hand, increased activity of Glc6Pase is thought to play an important role in hyperglycemia in type 1 and type 2
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