Results 1 to 10 of about 6,311 (212)

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, Liang Wang, Qinghua Liu, Junfeng Hu, James Asenso, Michael J. Wise, Michael J. Wise, Xiang Wu, Chao Ma, Xiuqing Chen, Jianye Yang, Daoquan Tang, Daoquan Tang   +12 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, Yiyang Li, Yiyang Li, Chuan Tian, Si Huang, Weijie Zhang, Weijie Zhang, Qiuyu Liutang, Qiuyu Liutang, Yajun Wang, Yajun Wang, Guoda Ma, Guoda Ma, Riling Chen, Riling Chen   +14 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, Mohd Basyaruddin Abdul Rahman, Raja Noor Zaliha Raja Abd Rahman, Thean Chor Leow, Abu Bakar Salleh, Mohd Noor Mat Isa   +5 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, Qiuxiang Ou, Pendleton Cox, Roland Lill, Kirst King-Jones   +4 more
doaj   +2 more sources

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, Jesús Casas, David J. López, Maitane Ibarguren, Ariadna Suari-Rivera, Silvia Terés, Francisca Guardiola-Serrano, Alexander Lossos, Xavier Busquets, Or Kakhlon, Pablo V. Escribá   +10 more
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Unifying the Communities of Early‐Onset Glycogen Storage Disease Type IV and Adult Polyglucosan Body Disease Through a Genetic Prevalence Study of GBE1‐Related Disease [PDF]

JIMD Reports
Glycogen storage disease type IV (GSD IV) is an autosomal recessive disorder caused by pathogenic variants in GBE1, resulting in deficient glycogen branching enzyme (GBE) activity and formation of abnormal glycogen (“polyglucosan”).
Rebecca L. Koch, H. Orhan Akman, Erin Chown, Deberah Goldman, Jeff Levenson, Qing Lu, Lindsay T. Michalovicz Gill, Matthew Morgan, Jennifer L. Orthmann‐Murphy, Natacha T. Pires, Rebecca Reef, Harriet Saxe, Moriel Singer‐Berk, Samantha Baxter   +13 more
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Stochastic modelling of a three-dimensional glycogen granule synthesis and impact of the branching enzyme.

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, Oliver Ebenhöh, Adélaïde Raguin   +2 more
doaj   +1 more source

The Structure of Maltooctaose-Bound Escherichia coli Branching Enzyme Suggests a Mechanism for Donor Chain Specificity

Molecules, 2023
Glycogen is the primary storage polysaccharide in bacteria and animals. It is a glucose polymer linked by α-1,4 glucose linkages and branched via α-1,6-linkages, with the latter reaction catalyzed by branching enzymes. Both the length and dispensation of
Remie Fawaz, Courtney Bingham, Hadi Nayebi, Janice Chiou, Lindsey Gilbert, Sung Hoon Park, James H. Geiger   +6 more
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Molecular basis of impaired glycogen metabolism during ischemic stroke and hypoxia. [PDF]

PLoS ONE, 2014
BACKGROUND: Ischemic stroke is the combinatorial effect of many pathological processes including the loss of energy supplies, excessive intracellular calcium accumulation, oxidative stress, and inflammatory responses.
Mohammed Iqbal Hossain, Carli Lorraine Roulston, David Ian Stapleton   +2 more
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Dysfunctional muscle and liver glycogen metabolism in mdx dystrophic mice. [PDF]

PLoS ONE, 2014
Duchenne muscular dystrophy (DMD) is a severe, genetic muscle wasting disorder characterised by progressive muscle weakness. DMD is caused by mutations in the dystrophin (dmd) gene resulting in very low levels or a complete absence of the dystrophin ...
David I Stapleton, Xianzhong Lau, Marcelo Flores, Jennifer Trieu, Stefan M Gehrig, Annabel Chee, Timur Naim, Gordon S Lynch, René Koopman   +8 more
doaj   +1 more source