Results 1 to 10 of about 38,476 (238)

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
doaj   +8 more sources

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   +5 more sources

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
doaj   +5 more sources

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
doaj   +5 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
doaj   +8 more sources

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.
Choi SY, Kang B, Choe JY, Lee Y, Jang HJ, Park HD, Lee SK, Choe YH.   +7 more
europepmc   +8 more sources

Structural basis of glycogen branching enzyme deficiency and pharmacologic rescue by rational peptide design. [PDF]

Hum Mol Genet, 2015
Glycogen branching enzyme 1 (GBE1) plays an essential role in glycogen biosynthesis by generating α-1,6-glucosidic branches from α-1,4-linked glucose chains, to increase solubility of the glycogen polymer.
Froese DS, Michaeli A, McCorvie TJ, Krojer T, Sasi M, Melaev E, Goldblum A, Zatsepin M, Lossos A, Álvarez R, Escribá PV, Minassian BA, von Delft F, Kakhlon O, Yue WW.   +14 more
europepmc   +7 more sources

Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase [PDF]

Journal of Neurochemistry, 2013
AbstractUncontrolled elongation of glycogen chains, not adequately balanced by their branching, leads to the formation of an insoluble, presumably neurotoxic, form of glycogen called polyglucosan. To test the suspected pathogenicity of polyglucosans in neurological glycogenoses, we have modeled the typical glycogenosis Adult Polyglucosan Body Disease ...
Or Kakhlon, Hava Glickstein, Naomi Feinstein, Yan Liu, Otto Baba, Tatsuo Terashima, Hasan O. Akman, Salvatore DiMauro, Alexander Lossos   +8 more
semanticscholar   +5 more sources

Prevalence of the Glycogen Branching Enzyme Deficiency Mutation in Quarter Horses in Brazil [PDF]

Journal of Equine Veterinary Science, 2017
Abstract Glycogen branching enzyme deficiency (GBED) is a fatal autosomal inherited disease affecting horses and caused by a nonsense mutation (c.102C>A) in the GBE1 gene. This disease strongly impairs the glycogen metabolism of affected animals resulting in abortion or early death of foals.
César Erineudo Tavares de Araújo, Diego José Zanzarini Delfiol, Peres Ramos Badial, José P. Oliveira‐Filho, João Pessoa Araújo Júnior, Alexandre Secorun Borges   +5 more
semanticscholar   +7 more sources

Glycogen Branching Enzyme Deficiency in Quarter Horse Foals [PDF]

Journal of Veterinary Internal Medicine, 2001
Seven related Quarter Horse foals that died by 7 weeks of age were examined for glycogen branching enzyme (GBE) deficiency. Clinical signs varied from stillbirth, transient flexural limb deformities, seizures, and respiratory or cardiac failure to persistent recumbency.
Stephanie J. Valberg, Tara L. Ward, Bonnie R. Rush, Hailu Kinde, Hajime Hiraragi, David Nahey, John C. Fyfe, James R. Mickelson   +7 more
  +6 more sources