Results 51 to 60 of about 38,476 (238)

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

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   +1 more source

Triacylglycerol mimetics regulate membrane interactions of glycogen branching enzyme: implications for therapy[S]

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 Álvarez, J. Casas, D. J. López, M. Ibarguren, Ariadna Suari-Rivera, Silvia Terés, F. Guardiola-Serrano, A. Lossos, X. Busquets, O. Kakhlon, P. Escribá   +10 more
semanticscholar   +1 more source

The heteromultimeric debranching enzyme involved in starch synthesis in Arabidopsis requires both isoamylase1 and isoamylase2 subunits for complex stability and activity.

PLoS ONE, 2013
Isoamylase-type debranching enzymes (ISAs) play an important role in determining starch structure. Amylopectin - a branched polymer of glucose - is the major component of starch granules and its architecture underlies the semi-crystalline nature of ...
Maria Sundberg, Barbara Pfister, Daniel Fulton, Sylvain Bischof, Thierry Delatte, Simona Eicke, Michaela Stettler, Steven M Smith, Sebastian Streb, Samuel C Zeeman   +9 more
doaj   +1 more source

GYS1 or PPP1R3C deficiency rescues murine adult polyglucosan body disease

Annals of Clinical and Translational Neurology, 2020
Objective Adult polyglucosan body disease (APBD) is an adult‐onset neurological variant of glycogen storage disease type IV. APBD is caused by recessive mutations in the glycogen branching enzyme gene, and the consequent accumulation of poorly branched ...
Erin E. Chown, Peixiang Wang, Xiaochu Zhao, Justin J. Crowder, Jordan W. Strober, Mitchell A. Sullivan, Yunlin Xue, Cody S. Bennett, Ami M. Perri, Bret M. Evers, Peter J. Roach, Anna A. Depaoli‐Roach, H. Orhan Akman, Bartholomew A. Pederson, Berge A. Minassian   +14 more
doaj   +1 more source

Analysis of Mesorhizobium loti Glycogen Operon: Effect of Phosphoglucomutase (pgm) and Glycogen Synthase (glgA) Null Mutants on Nodulation of Lotus tenuis

Molecular Plant-Microbe Interactions, 2002
The phosphoglucomutase (pgm) gene codes for a key enzyme required for the formation of UDP-glucose and ADP-glucose, the sugar donors for the biosynthesis of glucose containing polysaccharides.
Viviana C. Lepek, Alejandra L. D'Antuono, Pablo E. Tomatis, Juan E. Ugalde, Susana Giambiagi, Rodolfo A. Ugalde   +5 more
doaj   +1 more source

A Broad Characterization of Glycogen Storage Disease IV Patients: A Clinical, Genetic, and Histopathological Study

Biomedicines, 2023
Glycogen storage disease type IV (GSD IV) is an ultra-rare autosomal recessive disease caused by variants in the GBE1 gene, which encodes the glycogen branching enzyme (GBE). GSD IV accounts for approximately 3% of all GSD. The phenotype of GSD IV ranges
Matheus Vernet Machado Bressan Wilke, Bibiana Mello de Oliveira, Rodrigo Tzovenos Starosta, Marwan Shinawi, Liang Lu, Mai He, Yamin Ma, Janis Stoll, Carolina Fischinger Moura de Souza, Ana Cecilia Menezes de Siqueira, Sandra Maria Gonçalves Vieira, Carlos Thadeu Cerski, Lilia Farret Refosco, Ida Vanessa Doederlein Schwartz   +13 more
doaj   +1 more source

From omics to AI—mapping the pathogenic pathways in type 2 diabetes

FEBS Letters, EarlyView.
Integrating multi‐omics data with AI‐based modelling (unsupervised and supervised machine learning) identify optimal patient clusters, informing AI‐driven accurate risk stratification. Digital twins simulate individual trajectories in real time, guiding precision medicine by matching patients to targeted therapies.
Siobhán O'Sullivan, Lu Qi, Pierre Zalloua   +2 more
wiley   +1 more source

Cardiac Involvement in Glycogen Storage Disease Type IV: Two Cases and the Two Ends of a Spectrum

Case Reports in Medicine, 2012
Glycogen storage disease type IV (GSD IV) is an autosomal recessive disorder due to the deficiency of α 1,4-glucan branching enzyme, resulting in an accumulation of amylopectin-like polysaccharide in various systems.
Tolga Aksu, Ayse Colak, Omac Tufekcioglu
doaj   +1 more source

Plasma lipidomic and metabolomic profiles in high‐grade glioma patients before and after 72‐h presurgery water‐only fasting

Molecular Oncology, EarlyView.
Presurgery 72‐h fasting in GB patients leads to adaptations of plasma lipids and polar metabolites. Fasting reduces lysophosphatidylcholines and increases free fatty acids, shifts triglycerides toward long‐chain TGs and increases branched‐chain amino acids, alpha aminobutyric acid, and uric acid.
Iris Divé, Lisa Hahnefeld, Katharina J. Wenger, Donat Kögel, Joachim Steinbach, Gerd Geisslinger, Michael W. Ronellenfitsch, Irmgard Tegeder   +7 more
wiley   +1 more source

Expression of E. coli glycogen branching enzyme in an Arabidopsis mutant devoid of endogenous starch branching enzymes induces the synthesis of starch-like polyglucans

bioRxiv, 2015
Starch synthesis requires several enzymatic activities including branching enzymes (BEs) responsible for the formation of α(1→6) linkages. Distribution and number of these linkages are further controlled by debranching enzymes (DBEs) that cleave some of ...
Laura Boyer, X. Roussel, A. Courseaux, O. M. Ndjindji, C. Lancelon-Pin, J. Putaux, I. Tetlow, M. Emes, B. Pontoire, C. D’Hulst, F. Wattebled   +10 more
semanticscholar   +2 more sources