| glycogen i. |
abnormal accumulations of glycogen within the cytoplasm of cells, as occurs in diabetes mellitus and the glycogen storage diseases.
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| glycogen phosphorylase |
[EC 2.4.1.1] an enzyme of the transferase class that catalyzes the phosphorolysis of a terminal α-1,4-glycosidic bond at the non-reducing end of a glycogen molecule, releasing a glucose 1-phosphate residue. The inactive form of the enzyme (phosphorylase b) is converted to the active, phosphorylated form (phosphorylase a) by phosphorylase kinase, and phosphorylase a is deactivated by phosphorylase phosphatase; phosphorylase b can also be activated by AMP without being phosphorylated. Two isozymes exist: the liver isozyme replenishes blood glucose, while the muscle isozyme mobilizes glycogen as fuel. Deficiency of glycogen phosphorylase, an autosomal recessive trait, causes glycogen storage disease. The muscle isozyme is absent in glycogen storage disease, type V; the liver isozyme is deficient in type VI. Called also phosphorylase.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| glycogen phosphorylase kinase |
phosphorylase kinase.
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| glycogen storage d. |
any of a number of rare inborn errors of metabolism caused by defects in specific enzymes or transporters involved in the metabolism of glycogen.
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| glycogen synthase |
see glycogen starch synthase.
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