Glycogen branching enzyme

1,4- α -glucan branching enzyme ( GBE) is that enzyme poly -glucose chain adds to long-chain polysaccharides, but not to the end of the chain, but branching, such as at a distance 10 to 14 glucose units to the next branch (see figure). This reaction is (along with the extension of the chain), the final structure of the glycogen. The branches are needed to increase the solubility of glycogen, and therefore do not become too high osmotic pressure in the cell. GBE is found in most living organisms, the plant enzyme has the names of amylopectin branching enzyme or Q- enzyme. In humans, is located in particular in the liver and muscles. Mutations at GBE1 gene can lead to glycogen storage disease type IV ( Andersen 's disease ), or adults Polyglucosankörper disease.

Catalyzed reaction

The linear Glycogenkette is branched (similar in plants: amylose is converted to amylopectin).

Human Genome Organisation Glycosyltransferase
9045
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