1984 A direct evidence for defect in glucose-6-phosphate transport system in hepatic microsomal membrane of glycogen storage disease type 1b. Biochem Biophys Res Commun. 119:593-597.Igarashi Y, Kato S, Narisawa K, Tada K, Amano Y, Mori T, Takeuchi S (1984) A direct evidence for defect ...
Glycogen storage disease type I (GSDI) is a group of rare inherited diseases resulting from a defect in the glucose-6-phosphatase (G6Pase) system which has a key role in glucose homeostasis as it is required for the hydrolysis of glucose-6-phosphate (G6P) into glucose and inorganic phosph...
Conversion of glucose 6-phosphate to glucose 1-phosphate byphosphoglucomutaseprovides for interchange between glycogen,galactose, anduronic acidmetabolism (seeChapters 8and9). First, glucose 1-phosphate is activated to theuridine diphosphate(UDP) precursor, which then contributes to glycogen polymerization,...
Glycogen storage disease type I (GSDI) is a group of rare inherited diseases resulting from a defect in the glucose-6-phosphatase (G6Pase) system which has a key role in glucose homeostasis as it is required for the hydrolysis of glucose-6-phosphate (G6P) into glucose and inorganic phosph...
Two main sub- types are unambiguously recognized: GSD type Ia (GSDIa) due to a defect of the catalytic unit G6Pase- alpha (or G6PC), and GSD type Ib (GSDIb) due to a defect of the glucose-6-phosphate translocase (or G6PT) [1,2]. The existence of other types (type Ic and ...
They claimed that children with this disease, and their parents, have elevated levels of glucose6-phosphate and fructose-6-phosphate in their red blood cells. These findings have since been cited in other publications.2,3 If correct, this observation would have very important practical as well ...
Unfortunately, the use of 8-aminoquinolines is compromised by the potential haemolysis in individuals with the enzyme deficiency of glucose-6-phosphate dehydrogenase (G6PD). In consequence, enzyme activity testing of G6PD is essential before the administration of 8-aminoquinolines. Affecting over 400 ...
glucose transport maximum glucose/galactose malabsorption glucose-1-phosphate phosphodismutase glucose-1-phosphate uridylyltransferase glucose-6-phosphatase glucose-6-phosphate glucose-6-phosphate dehydrogenase glucose-6-phosphate dehydrogenase (G6PD) deficiency glucose-6-phosphate dehydrogenase deficiency glucose-6-...
The GLUT family of transporters may constitute the main entry route for glucosamine into the cell, and both GLUT1 and GLUT4 have been shown to transport glucosamine with similar kinetics [244]. However, as glucosamine is mainly produced endogenously from glucose via fructose-6-phosphate through th...
Glucose-6-phosphatase activity was normal in an aqueous homogenate (5.5 umol/gm/min), but had a latency of 78% in a sucrose homogenate (normal <10%, suggesting a defect in membrane transport of glucose-6-phosphate.Feeding a glucose-containing formula every 4 hours for the next year ...