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glycogen storage disease type Ia A form of glycogen storage disease with onset usually in the first year of life. This autosomal recessive genetic disorder is due to a glucose-6-phosphatase deficiency. SYN: von Gierke disease.
Ãâó:
glycogen storage disease type Ib A form of glycogen storage disease similar to type Ia but occurring at only one tenth its frequency. The disorder is due to a deficiency of glucose-6-phosphatase microsomal translocase.
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glycogenic infiltration Glycogen deposit in cells.
Ãâó:
glycogenv storage disease type VII A form of glycogen storage disease caused by a deficiency of muscle phosphofructokinase and characterized by muscular weakness and cramping following exercise. SEE: phosphofructokinase; Tarui disease
Ãâó:
glycogen (starch) synthase [EC 2.4.1.11] an enzyme of the transferase class that catalyzes the synthesis of glycogen by forming an α-1,4-glucoside linkage between the terminal hydroxyl group of glycogen chain and a glucosyl group donated by UDPglucose. The reaction is highly regulated by allosteric effectors, by kinases and phosphatases, and by insulin. The active form glycogen synthase a (formerly I) is not phosphorylated; the less active form glycogen synthase b (formerly D) is phosphorylated. The recommended name varies with the source of the enzyme; the human form is called glycogen synthase.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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