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Von Gierke disease

Excessive storage. Suggest an explanation for the fact that the amount of glycogen in type I glycogen-storage disease (von Gierke disease) is increased. [Pg.891]

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

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]

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. [Pg.80]

Blood glucose levels in patients with von Gierke disease fall precipitously upon fasting, such as occurs overnight during sleep, so treatment is to eat meals often to prevent hypoglycemic coma. [Pg.80]

TYPE la VON GIERKE DISEASE [GLUCOSE 6-PHOSPHATASE DEFICIENCY) Type lb GLUCOSE 6-PHOSPHA TE e TRANSLOCASE DEFICIENCY... [Pg.129]

Cause and result of glycogen storage disease type la (Von Gierke disease)... [Pg.479]

Carbohydrate abnormalities, such as renal glycosuria (a transport defect), pentosuria (enzyme deficiency, xylitol dehydrogenase I. lactase deficiencies, fructose intolerance, galactosemia, galacloki-nase deficiency, oxalosis, and several glycogenoses (von Gierke s, Forbes . Andersen s, Hers s. and Tarui s diseases). [Pg.716]

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]

Edgar von Gierke described the first glycogen-storage disease in 1929. A patient with this disease has a huge... [Pg.884]

See other pages where Von Gierke disease is mentioned: [Pg.227]    [Pg.461]    [Pg.876]    [Pg.890]    [Pg.615]    [Pg.227]    [Pg.461]    [Pg.876]    [Pg.890]    [Pg.615]    [Pg.750]    [Pg.300]    [Pg.388]    [Pg.61]    [Pg.195]    [Pg.478]    [Pg.133]    [Pg.134]    [Pg.299]    [Pg.479]    [Pg.495]    [Pg.568]    [Pg.270]    [Pg.484]    [Pg.110]    [Pg.884]    [Pg.884]   
See also in sourсe #XX -- [ Pg.387 ]



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