Glucose-6- phosphatase in liver

Comparison of Levels of Phosphotransferase and Phosphohydrolase Activities of Glucose-6-Phosphatase in Liver, Kidney, and Small Intestine of Rabbit"... 

The most common glycogen storage disease. Type I or von Gierke disease, is a deficiency in glucose 6-phosphatase in which glycogen structures are normal however, the liver is unable to dephosphory-late glucose 6-phosphate, and it remains trapped In the cell. 

Brondeau MT, Coulais C, de Ceaurriz J. 1991. Difference in liver and serum malathion carboxylesterase and glucose-6-phosphatase in detecting carbon tetrachloride-induced liver damage in rats. J AppI Toxicol 11 433-435. 

Reported Presence of Glucose-6-phosphatase in Vertebrate Liver... 

In the liver cell, the enzymes are spatially separated Glucokinase in the cytosol and glucose-6-phosphatase in the endoplasmic reticulum. Does this separation influence the futile cycle ... 

Ilyin, V.S. and Titova, G.V. (1963). In vitro effect of insulin on the activity of liver glucose-6-phosphate dehydrogenase and glucose-6-phosphatase (In Russian). Biochimika 28,987-991. 

Benedetti A, Fulceri R, Ferro M, Comporti M. On a possible role for glucose-6-phosphatase in the regulation of liver cell cytosolic calcium concentration. Trends Biochem. Sci. 1986 11 284-285. 

Diagnosis may be confirmed by the absence of glucose 6-phosphatase on liver biopsy. The liver cells are full of glycogen and fat. Also, the normal elevation in blood glucose from infusion of glucagon, epinephrine or galactose is absent. 

Brandt, K., Cathcart, E. S., and Cohen, A. S., A clinical analysis of the cause and prognosis of forty-two patients with amyloidosis. Am. ]. Med. 44, 955-969 (1968). Brante, G., Kaijser, K., and Ockerman, P. A., Glycogenosis Type I (lack of glucose-6-phosphatase) in four siblings. Acta Paediat. Scand. Suppl. 157, 1-28 (1964). Brensilver, H. L., and Kaplan, M. M., SigniRcance of elevat liver alkaline phosphatase in serum. Gastroenterology 68, 1556-1562 (1975). 

Explain the importance of glucose 6-phosphatase in the release of glucose by the liver. Note the absence of this enzyme in the brain and muscle. 

The fate of glucose-6-phosphatase in newborn liver has been investigated in some detail in several laboratories. The enzyme is absent from fetal liver of rodents and monkeys, but enzyme activity appears soon after birth by de novo synthesis of the enzyme molecules. Ethionine and puromycin, but not X-irradiation, pre-... 

Recent electronmicrographs illustrate the location of the electron-dense product of the hydrolytic activity of glucose-6-phosphatase in sections of rat liver (Wachstein and Meisel, 1956 Leskes et al., 1971 Rosen, 1969, 1970 DeMan, 1964 Orrenius and Ericsson, 1966) and of rat and mouse intestine (Hugon et al, 1970, 1971) and other tissues (Lazarus... 

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