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Glycogen debranching enzyme: Wikis

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amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III)
Identifiers
Symbol AGL
Entrez 178
HUGO 321
OMIM 232400
RefSeq NM_000028
UniProt P35573
Other data
EC number 2.4.1.25
Locus Chr. 1 p21

A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen.

Contents

Function

When a glycogen branch has only 4 glucose molecules left on that branch, the enzyme Phosphorylase stops working and the debranching enzyme takes over. Debranching enzymes are responsible for 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 alpha-1,6 glucosidase site of the debranching enzyme. This two step process is the general process by which the debranching enzymes "straighten 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.

Enzyme classification

The two debranching enzymes are:

Pathology

Deficiency in either of these enzymes will result in Glycogen storage disease type III.

External links

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