glycogen branching enzyme |
Identifiers |
EC number |
2.4.1.18 |
CAS number |
9001-97-2 |
Databases |
IntEnz |
IntEnz view |
BRENDA |
BRENDA entry |
ExPASy |
NiceZyme view |
KEGG |
KEGG entry |
MetaCyc |
metabolic pathway |
PRIAM |
profile |
PDB structures |
RCSB PDB PDBe PDBsum |
Gene Ontology |
AmiGO / EGO |
Search |
PMC |
articles |
PubMed |
articles |
NCBI Protein |
search |
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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 that takes part in converting glucose to glycogen. It adds branches to the growing glycogen molecule.
Contents
- 1 Function
- 2 Nomenclature
- 3 Pathology
- 4 Structural studies
- 5 References
- 6 External links
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Function
Glycogen is a branching polymer of large numbers of glucose units linked together. The structure is based on chains of glucose units with α(1→4) glycosidic bonds between carbon atoms 1 and 4 of each pair of units. 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 α(1→6) glycosidic bond. This connection is catalyzed by a branching enzyme. 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.
Nomenclature
This enzyme belongs to the family of transferases, to be specific, 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.
Structural studies
As of late 2007, only one structure has been solved for this class of enzymes, with the PDB accession code 1M7X.
References
- Barker SA, Bourne E and Peat S (Lond.). "The enzymic synthesis and degradation of starch. Part IV. The purification and storage of the Q-enzyme of the potato". J. Chem. Soc.: 1705–1711.
- Baum H and Gilbert GA (1953). "A simple method for the preparation of crystalline potato phosphorylase and Q-enzyme". Nature 171 (4361): 983–984. doi:10.1038/171983a0. PMID 13063502.
- Hehre EJ (1951). Enzymic Synthesis of Polysaccharides: a Biological type of Polymerization. "Advances in Enzymology and Related Areas of Molecular Biology". Adv. Enzymol. Relat. Subj. Biochem.. Advances in Enzymology - and Related Areas of Molecular Biology 11: 297–337. doi:10.1002/9780470122563.ch6. ISBN 978-0-470-12256-3.
- Handelsman DJ; Wang, Y; Jimenez, M; Marshan, B; Spaliviero, J; Illingworth, P; Handelsman, DJ (2006). "Follicle-stimulating hormone increases primordial follicle reserve in mature female hypogonadal mice". J. Endocrinol. 188 (3): 549–57. doi:10.1677/joe.1.06614. PMID 16522734.
External links
- GeneReviews/NCBI/NIH/UW entry on Adult Polyglucosan Body Disease
- OMIM entries on Adult Polyglucosan Body Disease
Transferases: glycosyltransferases (EC 2.4)
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2.4.1: Hexosyl-
transferases |
Glucosyl- |
- Phosphorylase
- Glycogen synthase
- Debranching enzyme
- Branching enzyme
- 1,3-beta-glucan synthase
- Ceramide glucosyltransferase
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Galactosyl- |
- Lactose synthase
- B-N-acetylglucosaminyl-glycopeptide b-1,4-galactosyltransferase
- Glycoprotein-N-acetylgalactosamine 3-beta-galactosyltransferase (C1GALT1)
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Glucuronosyl- |
- UGT1A1
- UGT1A3
- UGT1A4
- UGT1A5
- UGT1A6
- UGT1A7
- UGT1A8
- UGT1A9
- UGT1A10
- UGT2A1
- UGT2A2
- UGT2A3
- UGT2B4
- UGT2B7
- UGT2B10
- UGT2B11
- UGT2B15
- UGT2B17
- UGT2B28
- Hyaluronan synthase: HAS1
- HAS2
- HAS3
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Fucosyl- |
- POFUT1
- POFUT2
- FUT1
- FUT2
- FUT3
- FUT4
- FUT5
- FUT6
- FUT7
- FUT8
- FUT9
- FUT10
- FUT11
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Mannosyl- |
- Dolichyl-phosphate-mannose-protein mannosyltransferase
- DPM1
- DPM3
- ALG1
- ALG2
- ALG3
- ALG6
- ALG8
- ALG9
- ALG12
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2.4.2: Pentosyl-
transferases |
Ribose |
ADP-ribosyltransferase |
- NAD+:diphthamide ADP-ribosyltransferase
- NAD(P)+:arginine ADP-ribosyltransferase
- Pertussis toxin
- Cholera toxin
- Poly ADP ribose polymerase
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Phosphoribosyltransferase |
- Adenine phosphoribosyltransferase
- Hypoxanthine-guanine phosphoribosyltransferase
- Uracil phosphoribosyltransferase
- Amidophosphoribosyltransferase
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Other |
- Purine nucleoside phosphorylase: Thymidine phosphorylase
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Other |
- Xylosyltransferase
- Arabinosyltransferase
- Indolylacetylinositol arabinosyltransferase
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2.4.99: Sialyl
transferases |
- Beta-galactoside alpha-2,6-sialyltransferase
- Monosialoganglioside sialyltransferase
- ST8SIA4
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- B
- enzm
- 1.1
- 2
- 3
- 4
- 5
- 6
- 7
- 8
- 10
- 11
- 13
- 14
- 15-18
- 2.1
- 2.7.10
- 2.7.11-12
- 3.1
- 4.1
- 5.1
- 6.1-3
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Metabolism: carbohydrate metabolism · glycogenesis and glycogenolysis enzymes
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Glycogenesis |
Phosphoglucomutase · UDP-glucose pyrophosphorylase · Glycogen synthase · Glycogen branching enzyme
Glycogenin
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Glycogenolysis |
extralysosomal: Glycogen phosphorylase · Debranching enzyme · Phosphoglucomutase
lysosomal: Alpha-glucosidase (Acid)
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Regulation |
Phosphorylase kinase · Phosphoprotein phosphatase
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mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m
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k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon
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m(A16/C10),i(k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)
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