Human microsomal glucose-6-phosphatase system.
The discovery of glucose-6-phosphatase (EC 3.1.3.9) and of its physiological function in releasing glucose from the liver are discussed briefly. The identification by the Coris of glucose-6-phosphatase deficiency as the underlying defect in certain cases of glycogenosis (type I glycogenosis; von Gierke disease) is described. Characteristics of the catalyst, with a focus on its multiplicity of functions and multicomponent character, are considered with an emphasis on the human liver enzyme. Pioneering studies from the author's laboratory leading to the characterization of two variants of type I glycogenosis, types Ib and Ic, are described.
Nordlie
R C
RC
Department of Biochemistry and Molecular Biology, University of North Dakota School of Medicine, Grand Forks 58202.
Sukalski
K A
KA
Johnson
W T
WT
D005952:Glucose-6-Phosphatase / Q000378:metabolism*; D005953:Glycogen Storage Disease Type I / Q000145:classification / Q000201:enzymology* / Q000503:physiopathology; D006801:Humans; D008862:Microsomes, Liver / Q000201:enzymology*
D005952:Glucose-6-Phosphatase