Glycogen branching enzyme

From Wikipedia, the free encyclopedia

1,4-alpha-glucan branching enzyme
Identifiers
Symbol GBE1
Entrez 2632
HUGO 4180
OMIM 607839
RefSeq NM_000158
UniProt Q04446
Other data
EC number 2.4.1.18
Locus Chr. 3 p12

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.

Glycogen structure
Glycogen structure

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 glycosidic bond. 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.

This enzyme belongs to the family of transferases, specifically those glycosyltransferases that transfer hexoses (hexosyltransferases). The systematic name of this enzyme class is 1,4-alpha-D-glucan:1,4-alpha-D-glucan 6-alpha-D-(1,4-alpha-D-glucano)-transferase. Other names in common use include branching enzyme, amylo-(1,4→1,6)-transglycosylase, Q-enzyme, alpha-glucan-branching glycosyltransferase, amylose isomerase, enzymatic branching factor, branching glycosyltransferase, enzyme Q, glucosan transglycosylase, 1,4-alpha-glucan branching enzyme, plant branching enzyme, alpha-1,4-glucan:alpha-1,4-glucan-6-glycosyltransferase, and starch branching enzyme. This enzyme participates in starch and sucrose metabolism.

Contents

[edit] Pathology

A defect in this enzyme can lead to disease; see glycogen storage disease type IV and glycogen branching enzyme deficiency.

[edit] Structural studies

As of late 2007, only one structure has been solved for this class of enzymes, with the PDB accession code 1M7X.

[edit] References

[edit] External links

The CAS registry number for this enzyme class is 9001-97-2.

[edit] Gene Ontology (GO) codes