The glucose-6-phosphatase system in rat hepatic microsomes displays hyperbolic kinetics at physiological glucose 6-phosphate concentrations.

The glucose-6-phosphatase system.

Glucose-6-phosphatase (G6Pase), an enzyme found mainly in the liver and the kidneys, plays the important role of providing glucose during starvation. Unlike most phosphatases acting on water-soluble compounds, it is a membrane-bound enzyme, being associated with the endoplasmic reticulum. In 1975, W. Arion and co-workers proposed a model according to which G6Pase was thought to be a rather unsp...

Microsomal Membrane Permeability and the Hepatic Glucose-6-phosphatase System

We have proposed that glucose-6.phosphatase (EC 3.1.3.9) is a two-component system consisting of (a) a glucose-6-P-specific transporter which mediates the movement of the hexose phosphate from the cytosol to the lumen of the endoplasmic reticulum (or cisternae of the isolated microsomal vesicle), and (b) a nonspecific phosphohydrolase-phosphotransferase localized on the luminal surface of the m...

The specificity of glucose 6-phosphatase of intact liver microsomes.

Glucose-6-phosphatase deficiency

Glucose-6-phosphatase deficiency (G6P deficiency), or glycogen storage disease type I (GSDI), is a group of inherited metabolic diseases, including types Ia and Ib, characterized by poor tolerance to fasting, growth retardation and hepatomegaly resulting from accumulation of glycogen and fat in the liver. Prevalence is unknown and annual incidence is around 1/100,000 births. GSDIa is the more f...

Glucose-6-Phosphate Dehydrogenase

Description Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is the most common human enzyme deficiency that mainly affects the red blood cells (RBCs). A defect in G6PD enzyme leads to the destruction of premature RBCs causing hemolytic anemia because the body can not compensate the destroyed RBCs. Thus, the affected individuals show jaundice (paleness, yellowing of the skin and whites of th...