1,4-alpha-glucan branching enzyme activity (using a glucosylated glycogenin as primer for glycogen synthesis)
Cellular component
cytosol
extracellular exosome
Biological process
glycogen metabolic process
generation of precursor metabolites and energy
glycogen biosynthetic process
carbohydrate metabolic process
Sources:Amigo / QuickGO
Orthologs
Species
Human
Mouse
Entrez
2632
74185
Ensembl
ENSG00000114480
ENSMUSG00000022707
UniProt
Q04446
Q9D6Y9
RefSeq (mRNA)
NM_000158
NM_028803
RefSeq (protein)
NP_000149
NP_083079
Location (UCSC)
Chr 3: 81.49 – 81.76 Mb
Chr 16: 70.11 – 70.37 Mb
PubMed search
[3]
[4]
Wikidata
View/Edit Human
View/Edit Mouse
Glycogen branching enzyme
Identifiers
EC no.
2.4.1.18
CAS no.
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 / QuickGO
Search
PMC
articles
PubMed
articles
NCBI
proteins
1,4-alpha-glucan-branching enzyme, also known as brancher enzyme or glycogen-branching enzyme is an enzyme that in humans is encoded by the GBE1 gene.[5]
Glycogen branching enzyme is an enzyme that adds branches to the growing glycogen molecule during the synthesis of glycogen, a storage form of glucose. More specifically, during glycogen synthesis, a glucose 1-phosphate molecule reacts with uridine triphosphate (UTP) to become UDP-glucose, an activated form of glucose. The activated glucosyl unit of UDP-glucose is then transferred to the hydroxyl group at the C-4 of a terminal residue of glycogen to form an α-1,4-glycosidic linkage, a reaction catalyzed by glycogen synthase. Importantly, glycogen synthase can only catalyze the synthesis of α-1,4-glycosidic linkages. Since glycogen is a readily mobilized storage form of glucose, the extended glycogen polymer is branched by glycogen branching enzyme to provide glycogen breakdown enzymes, such as glycogen phosphorylase, with many terminal residues for rapid degradation. Branching also importantly increases the solubility and decreases the osmotic strength of glycogen.[6]
The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. The highest levels of this enzyme are found in liver and muscle cells. Mutations in this gene are associated with glycogen storage disease type IV (also known as Andersen's disease).
^ abcGRCh38: Ensembl release 89: ENSG00000114480 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000022707 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
4-alpha-glucan-branchingenzyme, also known as brancherenzyme or glycogen-branchingenzyme is an enzyme that in humans is encoded by the GBE1 gene. Glycogen branching...
the interior of the glycogen molecule. The branchingenzyme can act upon only a branch having at least 11 residues, and the enzyme may transfer to the...
Valberg, Stephanie; James R Mickelson. "GlycogenBranchingEnzyme Deficiency (GBED) in Horses". GlycogenBranchingEnzyme Deficiency (GBED). University of Minnsesota...
The glycogen debranching enzyme, in humans, is the protein encoded by the gene AGL. This enzyme is essential for the breakdown of glycogen, which serves...
breed's Glycogenbranchingenzyme (GBE1) can cause both a perinatal hypoglycemic collapse and a late-juvenile-onset neuromuscular degeneration in glycogen storage...
A glycogen storage disease (GSD, also glycogenosis and dextrinosis) is a metabolic disorder caused by a deficiency of an enzyme or transport protein affecting...
sequential removal of glucose monomers via phosphorolysis, by the enzymeglycogen phosphorylase. In the muscles, glycogenolysis begins due to the binding...
carbohydrates metabolism. Glycogen storage diseases are deficiencies of enzymes or transport proteins which impair glycogen synthesis, glycogen degradation or glycolysis...
for glycogenbranchingenzyme, also called 1,4-alpha-glucan-branchingenzyme. The GBE1 gene provides instructions for making the glycogenbranching enzyme...
Glycogen phosphorylase is one of the phosphorylase enzymes (EC 2.4.1.1). Glycogen phosphorylase catalyzes the rate-limiting step in glycogenolysis in...
control Genome Biology and Evolution, a scientific journal Glycogenbranchingenzyme, an enzyme The general balance equation, a simplified mass balance relation...
produced after hydrolysis of glycogen. Without glycogen debranching enzymes to further convert these branchedglycogen polymers to glucose, limit dextrinosis...
Quarter Horses and Appaloosas), malignant hyperthermia (MH) and glycogenbranchingenzyme deficiency (GBED). The influence of Thoroughbred breeding puts...
breakdown of glycogen to glucose-1-phosphate for use in muscle. The enzyme removes 1,4 glycosyl residues from outer branches of glycogen and adds inorganic...
extensively branched and compact than starch. Glycogen is a polymer of α(1→4) glycosidic bonds linked with α(1→6)-linked branches. Glycogen is found in...
intermediate and highly branched maltodextrins produced from gelatinized starches with various microbial glycogenbranchingenzymes". Carbohydrate Polymers...
an enzyme involved in converting glucose to glycogen. It acts as a primer, by polymerizing the first few glucose molecules, after which other enzymes take...
Isoamylase (EC 3.2.1.68, debranching enzyme, glycogen α-1,6-glucanohydrolase) is an enzyme with systematic name glycogen 6-α-D-glucanohydrolase. It catalyses...
the branchingenzyme in mycobacteria makes shorter branches compared to glycogen synthesis. For organisms that can utilize both classic glycogen synthesis...
core protein Glycogenin, surrounded by branches of glucose units, linked together. The branching of glycogen increases its solubility, and allows for...
extract different glycolytic enzymes from muscle tissue, and combine them to artificially create the pathway from glycogen to lactic acid. In one paper...
It is caused by a lack of activity in glycogen-branchingenzyme, resulting in accumulation of glycogen in the liver. Anderson never married and had no...
maltase enzymes, providing two glucose molecules that can be further processed: either broken down to provide energy, or stored as glycogen. The lack...
acids and glycogen. The metabolism of glycogen is controlled by activity of phosphorylase, the enzyme that breaks down glycogen, and glycogen synthase...
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