Glycogen branching enzyme
From Wikipedia, the free encyclopedia
A glycogen branching enzyme is an enzyme taking part in the synthesis of glycogen by adding branches to the glycogen molecule. Glycogen is a branching polymer of large numbers of glucose units linked together. The structure is based on chains of glucose units with linkages between carbon atoms 1 and 4 of each pair of units (alpha 1, 4 linkages). These linkages are catalyzed by the enzyme glycogen synthase.
Every 10 to 14 glucose units a side branch with an additional chain of glucose units occurs. The side chain attaches at carbon atom 6 of a glucose unit, and the linkage is termed an alpha 1, 6 link. To form this connection a separate enzyme known as a branching enzyme is used. A branching enzyme attaches a string of seven glucose units to the sixth carbon of a glucose unit, usually in an interior location of the glycogen molecule.
A defect in this enzyme can lead to disease; see glycogen storage disease type IV and glycogen branching enzyme deficiency.