Results 11 to 20 of about 6,177 (249)

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
openalex   +3 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
openalex   +3 more sources

Characterization of Two Glycoside Hydrolases of Family GH13 and GH57, Present in a Polysaccharide Utilization Locus (PUL) of Pontibacter sp. SGAir0037 [PDF]

Molecules
Glycogen, an α-glucan polymer serving as an energy storage compound in microorganisms, is synthesized through distinct pathways (GlgC-GlgA or GlgE pathway). Both pathways involve multiple enzymes, with a shared glycogen branching enzyme (GBE).
Hilda Hubertha Maria Bax, Edita Jurak
doaj   +2 more sources

Synthesis of highly branched α-glucans with different structures using GH13 and GH57 glycogen branching enzymes [PDF]

Carbohydrate Polymers, 2019
Glycogen branching enzymes (GBEs) convert starch into branched α-glucan polymers. To explore if the amylose content of substrates effects the structure of the branched α-glucans, mixtures of amylose and amylopectin were converted by four thermophilic GBEs.
Xuewen Zhang, Hans Leemhuis, Marc J. E. C. van der Maarel   +2 more
openalex   +4 more sources

Coordinate regulation of glycogen metabolism in the yeast Saccharomyces cerevisiae. Induction of glycogen branching enzyme.

Journal of Biological Chemistry, 1992
The yeast glycogen branching enzyme (EC 2.4.1.18) is shown to be induced in batch culture simultaneously with the onset of intracellular glycogen accumulation. The branching enzyme structural gene (GLC3) has been cloned. Its predicted amino acid sequence is very similar to procaryotic branching enzymes.
Vojtěch Thon, Corinne Vigneron-Lesens, T Marianne-Pepin, Jean Montreuil, André Decq, Christophe Rachez, Steven Ball, John F. Cannon   +7 more
openalex   +3 more sources

Neuromuscular forms of glycogen branching enzyme deficiency. [PDF]

Acta Myol, 2007
Deficiency of glycogen branching enzyme is causative of Glycogen Storage Disease type IV (GSD-IV), a rare autosomal recessive disorder of the glycogen synthesis, characterized by the accumulation of amylopectin-like polysaccharide, also known as polyglucosan, in almost all tissues.
Bruno C, Cassandrini D, Assereto S, Akman HO, Minetti C, Di Mauro S.   +5 more
europepmc   +3 more sources

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

Hum Mol Genet, 2015
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   +3 more sources

How did bacterial glycogen branching enzymes evolve? [PDF]

Research Outreach, 2021
Liang Wang, Qinghua Liu, Michael J. Wise, L Wang, M Abad, K Binderup, J Rios-Steiner, R Arni, J Preiss, J Geiger, A Waterhouse, M Bertoni, S Bienert, G Studer, G Tauriello, R Gumienny, F Heer, Ta De Beer, C Rempfer, L Bordoli   +19 more
openalex   +2 more sources

Identification of Thermotoga maritima MSB8 GH57 α-amylase AmyC as a glycogen-branching enzyme with high hydrolytic activity. [PDF]

Appl Microbiol Biotechnol, 2019
Zhang X, Leemhuis H, Janeček Š, Martinovičová M, Pijning T, van der Maarel MJEC.   +5 more
europepmc   +3 more sources

Influence of in situ progressive N-terminal is still controversial truncation of glycogen branching enzyme in Escherichia coli DH5α on glycogen structure, accumulation, and bacterial viability. [PDF]

BMC Microbiol, 2015
Wang L, Regina A, Butardo VM, Kosar-Hashemi B, Larroque O, Kahler CM, Wise MJ.   +6 more
europepmc   +3 more sources