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amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III)
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| 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
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. 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:
- 4-α-D-glucanotransferase (EC 2.4.1.25) - glucosyltransferase
- amylo-α-1,6-glucosidase (EC 3.2.1.33) - glucosidase
Pathology
Deficiency in either of these enzymes will result in Glycogen storage disease type III.
External links
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Wikipedia content modification information:
- This page was last modified on 3 October 2008, at 15:50.
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