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Gierke

Type 1 Von Gierke s disease Deficiency of glucose-6-phosphatase Liver cells and renal tubule cells loaded with glycogen. Hypoglycemia, lactic-acidemia, ketosis, hyperlipemia. [Pg.152]

Purine overproduction and hyperuricemia in von Gierke s disease (glucose-6-phosphatase deficiency)... [Pg.300]

Humans catabolize purines to uric acid (pA 5.8), present as the relatively insoluble acid at acidic pH or as its more soluble sodium urate salt at a pH near neutrality. Urate crystals are diagnostic of gout. Other disorders of purine catabolism include Lesch-Nyhan syndrome, von Gierke s disease, and hypo-uricemias. [Pg.301]

Siegrist RL, OR West, MI Morris, DA Pickering, DW Greene, CA Muhr, DD Davenport, JS Gierke (1995) In situ mixed region vapor stripping in low-permeability media. 2. Pull-scale field experiments. Environ Sci Technol 29 2198-2207. [Pg.618]

Inhibition of anion transport in Nation was attributed to the inhomogeneous structure of the ion exchange sites in the polymer network (Gierke cluster network model). It was found that Nation contains (even in... [Pg.143]

Fig. 2.20 The Gierke model of a cluster network in Nafion. Dimensions are expressed in nm. The shaded area is the double layer region, containing the immobilized —SO3 groups with corresponding number of counterions M+. Anions are expelled from this region electrostatically... Fig. 2.20 The Gierke model of a cluster network in Nafion. Dimensions are expressed in nm. The shaded area is the double layer region, containing the immobilized —SO3 groups with corresponding number of counterions M+. Anions are expelled from this region electrostatically...
Another class of carbohydrate and fatty acid metabolism disorders is caused by systemic metabolic defects that affect the brain. Glucose-6-phosphatase deficiency (glycogenosis type I, Von Gierke s disease)... [Pg.704]

B16. Bonsignore, A., Bulgarelli, R., Fornaini, G., and Fantoni, A., Comportamento di alcuni enzimi del metabolismo intermedio glucidico in un caso di morbo di Gierke. Proc. European Symp. Med. Enzymol., Milan, 1960 pp. 311-317 (1961). [Pg.297]

Carpenter, M. D., Gierke, J. S., et al., 1993, Vapor Phase Transport in Physically-Mixed Clay Soils In Proceedings of the 1993 Petroleum Hydrocarbons and Organic Chemicals in Ground Water Prevention, Detection and Restoration. American Petroleum Institute and National Ground Water Association, November 10-12, pp. 479-489. [Pg.324]

Gierke, T. D., Munn, G. E. and Wilson, F. C. 1981. The morphology in Nafion perfluorinated membrane products, as determined by wide-angle and small-angle x-ray studies. Journal of Polymer Science Polymer Physics 19 1687-1704. [Pg.171]

The earliest fully atomistic molecular dynamic (MD) studies of a simplified Nation model using polyelectrolyte analogs showed the formation of a percolating structure of water-filled channels, which is consistent with the basic ideas of the cluster-network model of Hsu and Gierke. The first MD... [Pg.359]

I von Gierke Glucose-6-phosphatase Severe hypoglycemia, lactic acidosis, hepatomegaly, hyperlipidemia, hyperuricemia, short stature Normal... [Pg.195]

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See also in sourсe #XX -- [ Pg.91 ]



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Disease, von Gierke

Gierke cluster network model

Gierke model

Gierke’s disease

Von Gierke’s disease

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