glycogen branching enzyme

• グリコーゲン分枝酵素

関

1,4-alpha-glucan branching enzyme、branching enzyme、starch branching enzyme

WordNet

1. one form in which body fuel is stored; stored primarily in the liver and broken down into glucose when needed by the body (同)animal_starch
2. resembling the branches of a tree
3. the act of branching out or dividing into branches (同)ramification, fork, forking
4. any of several complex proteins that are produced by cells and act as catalysts in specific biochemical reactions

PrepTutorEJDIC

1. グリコーゲン,糖原
2. 酵素

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/06/21 04:12:53」(JST)

wiki en

[Wiki en表示]

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 proteins

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.

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, generally given the name α-glucan branching enzyme. A branching enzyme attaches a string of seven glucose units (with some minor variation to this number) to the carbon at the C6 position on the glucose unit, forming the α(1→6) glycosidic bond. The specific nature of this enzyme means that this chain of 7 carbons is usually attached to a glucose molecule that is in position three from the non-reducing end of another chain. Because the enzyme works with such specificity regarding the number of glucose units transferred and the position to which they are transferred, the enzyme creates the very characteristic; highly-branched 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

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. グリコーゲン分枝酵素欠損症（糖原病IV型、アンダーセン病） glycogen branching enzyme deficiency glycogen storage disease iv andersen disease
• 2. グルコースおよびグリコーゲン代謝の遺伝性疾患の概要 overview of inherited disorders of glucose and glycogen metabolism
• 3. 新生児における筋緊張低下を引き起こす末梢神経および筋肉の障害に関する概要 overview of peripheral nerve and muscle disorders causing hypotonia in the newborn
• 4. 肝グリコーゲン合成酵素欠損症（糖原病0型） liver glycogen synthase deficiency glycogen storage disease 0
• 5. リソソーム酸α-グルコシダーゼ欠損症（ポンペ病、糖原病II型、酸マルターゼ欠損症） lysosomal acid alpha glucosidase deficiency pompe disease glycogen storage disease ii acid maltase deficiency

English Journal

• Adult Polyglucosan Body Disease presenting as a unilateral progressive plexopathy.

• Naddaf E1, Kassardjian CD1, Kurt YG2, Akman HO2, Windebank AJ1.
• Muscle & nerve.Muscle Nerve.2016 Jan 20. doi: 10.1002/mus.25041. [Epub ahead of print]
• INTRODUCTION: Adult polyglucosan body disease (APBD) usually presents with progressive spastic paraparesis, neurogenic bladder, and distal lower limb sensory abnormalities. It is caused by mutations in glycogen branching enzyme gene (GBE1).METHODS: We describe a woman with an unusual phenotype manif
• PMID 26789422

• Expression of E. coli glycogen branching enzyme in an Arabidopsis mutant devoid of endogenous starch branching enzymes induces the synthesis of starch-like polyglucans.

• Boyer L1, Roussel X1, Courseaux A1, Ndjindji OM2,3, Lancelon-Pin C2,3, Putaux JL2,3, Tetlow I4, Emes M4, Pontoire B5, D'Hulst C1, Wattebled F1.
• Plant, cell & environment.Plant Cell Environ.2015 Dec 30. doi: 10.1111/pce.12702. [Epub ahead of print]
• Starch synthesis requires several enzymatic activities including branching enzymes (BEs) responsible for the formation of α(1→6) linkages. Distribution and number of these linkages are further controlled by debranching enzymes (DBEs) that cleave some of them, rendering the polyglucan water-insolu
• PMID 26715025

• Altering the Structure of Carbohydrate Storage Granules in the Cyanobacterium Synechocystis sp. Strain PCC 6803 through Branching-Enzyme Truncations.

• Welkie DG1, Lee BH2, Sherman LA3.
• Journal of bacteriology.J Bacteriol.2015 Dec 14;198(4):701-10. doi: 10.1128/JB.00830-15.
• Carbohydrate storage is an important element of metabolism in cyanobacteria and in the chloroplasts of plants. Understanding how to manipulate the metabolism and storage of carbohydrate is also an important factor toward harnessing cyanobacteria for energy production. While most cyanobacteria produc
• PMID 26668264

Japanese Journal

• Purification and Characterization of a Novel Glycogen Branching Enzyme from <i>Paenibacillus</i> sp. SSG-1 and its Application in Wheat Bread Making

• Xu Qingrui,Cao Yu,Ma Xiaorui,Liu Lin,Wu Haizhen,Song Tao,Xu Hui,Qiao Dairong,Cao Yi
• Food Science and Technology Research 22(5), 655-664, 2016
• … <p>Glycogen branching enzyme contributes to the process of glycogen synthesis by creating α (1→6) branches through cleaving the α-1, 4-glycosidic bonds. … An intracellular glycogen branching enzyme (named PsGBE) from <i>Paenibacillus</i> … The recombinant enzyme was purified by metal-affinity chromatography, exhibited a molecular mass of 84.9 kDa. …
• NAID 130005268259

• Characterization of Starch and Glycogen Branching Enzymes from Various Sources

• Sawada Takayuki,Nakagami Tsuyoshi,Utsumi Yoshinori [他],OHDAN Takashi,SUZUKI Eiji,NAKAMURA Yasunori
• Journal of applied glycoscience 60(1), 69-78, 2013-02-20
• NAID 10031139757

• Characterization of Starch and Glycogen Branching Enzymes from Various Sources

• Sawada Takayuki,Nakagami Tsuyoshi,Utsumi Yoshinori,Ohdan Takashi,Suzuki Eiji,Nakamura Yasunori
• Ｊｏｕｒｎａｌ　ｏｆ　Ａｐｐｌｉｅｄ　Ｇｌｙｃｏｓｃｉｅｎｃｅ 60(1), 69-78, 2013
• … The present paper comparatively characterized starch and glycogen branching enzymes (BEs) from various organisms. …
• NAID 130004481016

Related Links

• Glycogen Branching Enzyme Deficiency | College of ...

Glycogen branching enzyme deficiency (GBED) is a disorder first recognized by clinicians at the University of Minnesota that causes muscle weakness in Quarter Horse and related breeds. The clinical presentation of this disease is ...

• Glycogen Branching Enzyme - an overview | ScienceDirect Topics

The glycogen branching enzyme (GBE) catalyzes the last step in glycogen biosynthesis by attaching a short glucosyl chain (about 7 glucosyl units) in an α-1,6-glucosidic link to a naked peripheral chain of nascent glycogen. The ...

• GLYCOGEN BRANCHING ENZYME; GBE1

Thon et al. (1993) used functional complementation of the Saccharomyces cerevisiae glycogen branching enzyme deficiency to isolate human cDNAs that encode the glycogen branching enzyme. The human and yeast glycogen ...

Related Pictures

---

★リンクテーブル★

リンク元	「branching enzyme」「starch branching enzyme」「1,4-alpha-glucan branching enzyme」「グリコーゲン分枝酵素」
拡張検索	「glycogen branching enzyme system」
関連記事	「branching enzyme」「branching」「glycogen」

「branching enzyme」

　　[★]

分枝酵素、枝分かれ酵素

関

1,4-alpha-glucan branching enzyme、glycogen branching enzyme、starch branching enzyme

「starch branching enzyme」

　　[★]

• デンプン分枝酵素

関

1,4-alpha-glucan branching enzyme、branching enzyme、glycogen branching enzyme

「1,4-alpha-glucan branching enzyme」

　　[★]

1

関

branching enzyme、glycogen branching enzyme、starch branching enzyme

「グリコーゲン分枝酵素」

　　[★]

英

glycogen branching enzyme

関

グリコーゲン

「glycogen branching enzyme system」

　　[★] グリコーゲン分枝酵素システム

「branching enzyme」

　　[★]

分枝酵素、枝分かれ酵素

関

1,4-alpha-glucan branching enzyme、glycogen branching enzyme、starch branching enzyme

「branching」

　　[★]

• n.

• 分岐、枝分かれ、分枝

　 　

「glycogen」

　　[★] グリコーゲン