Results 11 to 20 of about 1,002,705 (170)

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   +2 more sources

Characterization of the GH13 and GH57 glycogen branching enzymes from Petrotoga mobilis SJ95 and potential role in glycogen biosynthesis. [PDF]

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, Hans Leemhuis, Marc J E C van der Maarel   +2 more
doaj   +3 more sources

The Unique Branching Patterns of Deinococcus Glycogen Branching Enzymes Are Determined by Their N-Terminal Domains [PDF]

Applied and Environmental Microbiology, 2009
ABSTRACT Glycogen branching enzymes (GBE) or 1,4-α-glucan branching enzymes (EC 2.4.1.18) introduce α-1,6 branching points in α-glucans, e.g., glycogen. To identify structural features in GBEs that determine their branching pattern specificity, the Deinococcus geothermalis and Deinococcus ...
Palomo, M., Kralj, S., van der Maarel, M. J. E. C., Dijkhuizen, L.   +3 more
openaire   +6 more sources

The diversity of glycogen branching enzymes in microbes [PDF]

, 2019
Glycogen is the major carbon and energy reserve polymer in microorganisms and animals. Glycogen branching enzyme is one of the key enzymes in the glycogen synthesis. The glycogen branching enzymes were classified into GH13 and GH57 families. In this thesis, the two families GBEs were compared from crystal structures to their branched products. The GH13
Zhang, Xuewen
core   +17 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   +2 more sources

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
doaj   +1 more source

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
doaj   +1 more source

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

Glycogen Branching Enzyme Deficiency in Quarter Horse Foals [PDF]

Journal of Veterinary Internal Medicine, 2001
Abstract 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. Leukopenia (5 of 5 foals) as well as high serum creatine
S J, Valberg, T L, Ward, B, Rush, H, Kinde, H, Hiraragi, D, Nahey, J, Fyfe, J R, Mickelson   +7 more
openaire   +2 more sources

Alleviation of a polyglucosan storage disorder by enhancement of autophagic glycogen catabolism

EMBO Molecular Medicine, 2021
This work employs adult polyglucosan body disease (APBD) models to explore the efficacy and mechanism of action of the polyglucosan‐reducing compound 144DG11.
Or Kakhlon, Hilla Vaknin, Kumudesh Mishra, Jeevitha D’Souza, Monzer Marisat, Uri Sprecher, Shane Wald‐Altman, Anna Dukhovny, Yuval Raviv, Benny Da’adoosh, Hamutal Engel, Sandrine Benhamron, Keren Nitzan, Sahar Sweetat, Anna Permyakova, Anat Mordechai, Hasan Orhan Akman, Hanna Rosenmann, Alexander Lossos, Joseph Tam, Berge A. Minassian, Miguel Weil   +21 more
doaj   +1 more source