Triacylglycerol mimetics regulate membrane interactions of glycogen branching enzyme: implications for therapy [PDF]
Journal of Lipid Research, 2017 Adult polyglucosan body disease (APBD) is a neurological disorder characterized by adult-onset neurogenic bladder, spasticity, weakness, and sensory loss.
Rafael Alvarez +10 more
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Expression and characterization of thermostable glycogen branching enzyme from Geobacillus mahadia Geo-05 [PDF]
PeerJ, 2016 The glycogen branching enzyme (EC 2.4.1.18), which catalyses the formation of α-1,6-glycosidic branch points in glycogen structure, is often used to enhance the nutritional value and quality of food and beverages. In order to be applicable in industries,
Nur Syazwani Mohtar +5 more
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Structure and Evolution of Glycogen Branching Enzyme N-Termini From Bacteria [PDF]
Frontiers in Microbiology, 2019 In bacteria, glycogen plays important roles in carbon and energy storage. Its structure has recently been linked with bacterial environmental durability.
Liang Wang +12 more
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Glycogen branching enzyme controls cellular iron homeostasis via Iron Regulatory Protein 1 and mitoNEET [PDF]
Nature Communications, 2019 Higher organisms regulate cellular iron concentrations through Iron Regulatory Proteins (IRPs), which regulate specific messenger RNAs. Here Huynh et al.
Nhan Huynh +4 more
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Case report: Familial glycogen storage disease type IV caused by novel compound heterozygous mutations in a glycogen branching enzyme 1 gene [PDF]
Frontiers in Genetics, 2022 Glycogen storage disease type IV (GSD IV), caused by a mutation in the glycogen branching enzyme 1 (GBE1) gene, is a rare metabolic disorder with an autosomal recessive inheritance that involves the liver, neuromuscular, and cardiac systems.
Yiyang Li +14 more
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A Case of Glycogen Storage Disease IV with Rare Homozygous Mutations in the Glycogen Branching Enzyme Gene. [PDF]
Pediatr Gastroenterol Hepatol Nutr, 2018 Glycogen storage disease (GSD) IV is a rare autosomal recessive inherited disorder caused by mutations in the gene coding for glycogen branching enzyme leading to progressive liver disease. GSD IV is associated with mutations in GBE1, which encodes the glycogen branching enzyme. We report a case of GSD IV with rare homozygous mutations in the GBE1 gene
Choi SY +7 more
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PLoS ONE, 2019 Glycogen is a highly branched α-glucan polymer widely used as energy and carbon reserve by many microorganisms. The branches are introduced by glycogen branching enzymes (EC 2.4.1.18), that are classified into glycoside hydrolase families 13 (GH13) and ...
Xuewen Zhang +2 more
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PLoS Computational Biology, 2023 In humans, glycogen storage diseases result from metabolic inborn errors, and can lead to severe phenotypes and lethal conditions. Besides these rare diseases, glycogen is also associated to widely spread societal burdens such as diabetes.
Yvan Rousset +2 more
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Purification and Subunit Structure of Glycogen-Branching Enzyme from Rabbit Skeletal Muscle [PDF]
European Journal of Biochemistry, 1980 1,4-alpha-glucan:1,4-alpha-glucan 6-alpha-D-(1,4-alpha-D-glucano) transferase (branching enzyme) was purified by ammonium sulphate precipitation, chromatography on DEAE-cellulose, fractionation with poly(ethyleneglycol) 6000, chromatography on DEAE-Sepharose and gel filtration on Sephadex G150. The final specific activity was 3000 U/mg corresponding to
F. Barry Caudwell, Philip Cohen
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The versatile activity of glycogen branching enzymes [PDF]
, 2020 Glycogen branching enzymes (GBEs) are active in the biosynthesis of glycogen and can be applied to modify starch. These enzymes have been described to introduce new branch points but their exact mechanism is still little understood due to the size and complexity of both their substrate and product.
Lucie Gänssle
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