Glycogen branching enzyme
- Glycogen branching enzyme
protein
Name=1,4-alpha-glucan branching enzyme
caption=
width=
HGNCid=4180
Symbol=GBE1
AltSymbols=
EntrezGene=2632
OMIM=607839
RefSeq=NM_000158
UniProt=Q04446
PDB=
ECnumber=2.4.1.18
Chromosome=3
Arm=p
Band=12
LocusSupplementaryData=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 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.
Pathology
A defect in this enzyme can lead to disease; see glycogen storage disease type IV and glycogen branching enzyme deficiency.
tructural studies
As of late 2007, only one structure has been solved for this class of enzymes, with the PDB accession code PDB link|1M7X.
References
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External links
::"The CAS registry number for this enzyme class is CAS registry|9001-97-2."
Gene Ontology (GO) codes
Wikimedia Foundation.
2010.
Look at other dictionaries:
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