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Von Gierke s disease

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]

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]

I (lA and IB) Glucose-6-phosphatase von Gierke s disease Enlarged liver and kidney slowed growth very low blood sugar levels abnormally high levels of acid, fats and uric acid in blood growth failure... [Pg.111]

XI Glucose transporter, GLUT2 Fanconi-Bickel syndrome Similar to Von Gierke s disease, e.g. hypoglycaemia... [Pg.111]

D-2) Glucftse 6-phosphatase deficiency Cl pe I GSD Von Gierke s Disease). There is a deficiency at this step in the formation of glucose by the liver. Glucose 6-phosphate instead forms other things and the flow of reactions shifts to ... [Pg.49]

Percent conversion to a-n-glucopyranosyl phosphate by muscle phosphorylase this is a measure of the exterior chain length. This glycogen was incorrectly described as coming from a case of von Gierke s disease. ... [Pg.293]

A. Glucose 6-phosphatase deficiency is a glycogen storage disease (von Gierke s disease) in... [Pg.182]

QUESTION 8.6 Patients with Von Gierke s disease (a glycogen storage disease) lack glucose-6-phosphatase activity. Two prominent symptoms of this disorder are fasting hypoglycemia and lactic acidosis. Can you explain why these symptoms occur ... [Pg.255]

See other pages where Von Gierke s disease is mentioned: [Pg.750]    [Pg.300]    [Pg.388]    [Pg.61]    [Pg.478]    [Pg.568]    [Pg.270]    [Pg.484]    [Pg.110]    [Pg.843]    [Pg.876]    [Pg.890]    [Pg.890]    [Pg.292]    [Pg.212]    [Pg.236]    [Pg.198]    [Pg.348]    [Pg.349]    [Pg.352]    [Pg.654]    [Pg.654]    [Pg.209]    [Pg.40]    [Pg.691]   
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See also in sourсe #XX -- [ Pg.478 ]

See also in sourсe #XX -- [ Pg.270 ]

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