Glycobiology, 2021 Branching enzymes (BE) are responsible for the formation of branching points at the 1,6 position in glycogen and starch, by catalyzing the cleavage of α-1,4-linkages and the subsequent transfer by introducing α-1,6-linked glucose branched points. BEs are
Léa Conchou +7 more
semanticscholar +3 more sources
Glycogen storage disease due to glycogen branching enzyme deficiency [PDF]
Definitions, 2020 glycogen glycogen storage ...
openalex +2 more sources
Isolation of human glycogen branching enzyme cDNAs by screening complementation in yeast.
Journal of Biological Chemistry, 1993 Functional complementation of the Saccharomyces cerevisiae glycogen branching enzyme deficiency was screened to isolate human cDNAs that encode this enzyme. Human hepatoma cell line HepG2-derived cDNA libraries using the pAB23BXN yeast expression vector yielded four cDNAs capable of complementing the glc3::TRP1 glycogen branching enzyme mutation ...
Vojtěch Thon, M Khalil, John F. Cannon
openalex +4 more sources
Structural features of the glycogen branching enzyme encoding genes from aspergilli
Microbiological Research, 2002 A maltose binding protein, p78, was purified to homogeneity from Aspergillus nidulans by a single column chromatography step on cross-linked amylose. The partial amino acid sequence was highly homologous to the glycogen branching enzymes (GBEs) of human and yeast, and p78 did show branching enzyme activity.
Sasangka Prasetyawan +10 more
openalex +4 more sources
The diverse roles of glycogen branching enzymes and their synergistic activity [PDF]
Branched α-glucans like starch and glycogen are vital for storing energy in organisms. Glycogen accumulates when nutrients are scarce but carbon is available. Its structure, including chain length and branching, varies among bacteria and is crucial for its function.
Hilda Hubertha Maria Bax
openalex +3 more sources
A novel missense mutation in the glycogen branching enzyme gene in a child with myopathy and hepatopathy [PDF]
Neuromuscular Disorders, 1999 We have identified a novel missense mutation in the gene for glycogen branching enzyme (GBE 1) in a 16-month-old infant with a combination of hepatic and muscular features, an atypical clinical presentation of glycogenosis type IV (GSD IV). The patient was heterozygous for a G-to-A substitution at codon 524 (R524Q), changing an encoded arginine (CGA ...
Claudio Bruno +10 more
openalex +6 more sources
Clinical genetic analysis of an adult polyglucosan body disease (APBD) family caused by the compound heterozygous variant of GBE1 p.R156C and deletion exon 3-7 [PDF]
Frontiers in GeneticsIntroductionAdult Polyglucosan Body Disease (APBD) is a rare, autosomal recessive neurodegenerative disorder that affects both the central and peripheral nervous systems. It is primarily caused by mutations in the Glycogen Branching Enzyme 1 (GBE1) gene.
Juan Zhu +14 more
doaj +2 more sources
, 2016 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 Paenibacillus sp.
Qingrui Xu +8 more
openalex +3 more sources
Synthesis of Hyperbranched Glycoconjugates by the Combined Action of Potato Phosphorylase and Glycogen Branching Enzyme from Deinococcus geothermalis [PDF]
, 2012 Potato phosphorylase is able to synthesize linear polyglucans from maltoheptaose primers. By coupling maltoheptaose to butane diamine, tris(2-aminoethyl)amine and amine functionalized amine functionalized poly ethyleneglycol (PEG), new primer molecules ...
Jeroen van der Vlist +5 more
openalex +2 more sources