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A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen.
[edit] Function
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.
These enzymes are important in glycogenolysis because the cutting enzyme, glycogen phosphorylase, cannot cut a non-linear (or branched) glycogen chain.
[edit] Enzyme classification
The two debranching enzymes are:
[edit] Pathology
Deficiency in either of these enzymes will result in Glycogen storage disease type III.
[edit] External links
