Glycogen debranching enzyme
amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III)
|Locus||Chr. 1 p21|
A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen.
Debranching enzymes work by first transferring three glucose subunits of glycogen from one parallel chain to another. This shortens one linear branch while lengthening another. Afterwards, the donator branch will contain only one glucose residue with alpha-1,6 linkage. This remaining residue is in turn cut by the alpha-1,6 glucosidase activity of the debranching enzyme, and attached to the remaining linear branch. This two step process is the general mechanism by which the debranching enzyme "straightens out" glycogen into an unbranched glucose polymer.
The two debranching enzymes are:
- 4-α-D-glucanotransferase (EC 18.104.22.168) - glucosyltransferase
- amylo-α-1,6-glucosidase (EC 22.214.171.124) - glucosidase
Deficiency in either of these enzymes will result in Glycogen storage disease type III.
Metabolism: carbohydrate metabolism - glycogenesis and glycogenolysis enzymes
|Glycogenesis||Phosphoglucomutase - UDP-glucose pyrophosphorylase - Glycogen synthase (Glycogen branching enzyme)|
|Glycogenolysis||Glycogen phosphorylase (Debranching enzyme) - Phosphoglucomutase - Glycogenin|
|Regulation||Phosphorylase kinase - Phosphoprotein phosphatase|
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