HUMAN MICROSOMAL GLUCOSE-6-PHOSPHATASE SYSTEM

The discovery of glucose-6-phosphatase (EC 3.1.3.9) and of its physiol ogical function in releasing glucose from the liver are discussed brie fly. The identification by the Coris of glucose-6-phosphatase deficien cy as the underlying defect in certain cases of glycogenosis (type I g lycogenosis; von Gierke disease) is described. Characteristics of the catalyst, with a focus on its multiplicity of functions and multicompo nent character, are considered with an emphasis on the human liver enz yme. Pioneering studies from the author's laboratory leading to the ch aracterization of two variants of type I glycogenosis, types Ib and Ic , are described.