Publications related to Glycogen storage disease

Early Neuroprotective Effects of Bovine Lactoferrin Associated with Hypothermia after Neonatal Brain Hypoxia-Ischemia in Rats

Neonatal hypoxic-ischemic (HI) encephalopathy (HIE) in term newborns is a leading cause of mortality and chronic disability. Hypothermia (HT) is the only clinically available therapeutic intervention; however, its neuroprotective effects are limited. Lacto ...

MDPI2023

Investigation of physiological roles of AMPK-activated protein kinase y3

Philipp Jurijvic Rhein

Clinical trials have shown that direct activators of an evolutionary-conserved metabolic sensor, AMP-activated protein kinase (AMPK), are beneficial in preventing/treating a range of metabolic disorders, including type 2 diabetes. These activators, includi ...

EPFL2021

Glycogen metabolism is impaired in the brain of male type 2 diabetic Goto‐Kakizaki rats

João Miguel das Neves Duarte, Ana Francisca Leal Silva Soares

Diabetes impacts the central nervous system predisposing to cognitive decline. While glucose is the main source of energy fueling the adult brain, brain glycogen is necessary for adequate neuronal function, synaptic plasticity and memory. In this study, we ...

2019

Ultra high magnetic field for glial contribution into brain metabolism studied by MR spectroscopy and CEST methods for molecular imaging of glycogen

Elise Marie Catherine Vinckenbosch

Magnetic resonance at ultra-high field increases signal and spectral dispersion. In this thesis, I use those characteristics to investigate three different subjects in 13C spectroscopy, hyperpolarized methods and chemical exchange saturation transfer (CEST ...

EPFL2018

Glycogen Supercompensation in the Rat Brain After Acute Hypoglycemia is Independent of Glucose Levels During Recovery

Rolf Gruetter, João Miguel das Neves Duarte

Patients with diabetes display a progressive decay in the physiological counter-regulatory response to hypoglycemia, resulting in hypoglycemia unawareness. The mechanism through which the brain adapts to hypoglycemia may involve brain glycogen. We tested t ...

Springer Verlag2017

Technical and experimental features of Magnetic Resonance Spectroscopy of brain glycogen metabolism

Rolf Gruetter, Hongxia Lei, Ana Francisca Leal Silva Soares

In the brain, glycogen is a source of glucose not only in emergency situations but also during normal brain activity. Altered brain glycogen metabolism is associated with energetic dysregulation in pathological conditions, such as diabetes or epilepsy. Bot ...

Elsevier2017

Abnormal creatine transport of mutations in monocarboxylate transporter 12 (MCT12) found in patients with age-related cataract can be partially rescued by exogenous chaperone CD147

Membrane transporters influence biological functions in the ocular lens. Here, we investigate the monocarboxylate transporter 12 (MCT12), also called creatine transporter 2 (CRT2), which is found in the ocular lens and is involved in cataract. As the age-r ...

Oxford University Press2017

AMP-activated protein kinase : strategies for activation and downstream target identification

Serge Thanh Son Ducommun

AMP-activated protein kinase (AMPK) is an evolutionally conserved key sensor of cellular energy status. Under conditions of cellular energy stress, AMPK is activated and will switch off anabolic processes that consume ATP, while activating catabolic proces ...

EPFL2016

Three-dimensional immersive virtual reality for studying cellular compartments in 3D models from EM preparations of neural tissues

Pierre Magistretti, Corrado Cali, Daniya Boges, Madhusudhanan Srinivasan

Advances in the application of electron microscopy (EM) to serial imaging are opening doors to new ways of analyzing cellular structure. New and improved algorithms and workflows for manual and semiautomated segmentation allow us to observe the spatial arr ...

Wiley-Blackwell2016

Glutamate Cysteine Ligase-Modulatory Subunit Knockout Mouse Shows Normal Insulin Sensitivity but Reduced Liver Glycogen Storage

Pierre Magistretti, Kim Do

Glutathione (GSH) deficits have been observed in several mental or degenerative illness, and so has the metabolic syndrome. The impact of a decreased glucose metabolism on the GSH system is well-known, but the effect of decreased GSH levels on the energy m ...

Frontiers Media Sa2016

Regulation Of Neuron-Astrocyte Metabolic Coupling Across The Sleep-Wake Cycle

Pierre Magistretti, Jean-Marie Petit

Over the last thirty years, a growing number of studies showed that astrocytes play a pivotal role in the energy support to synapses. More precisely, astrocytes adjust energy production to neuronal energy needs through different mechanisms grouped under th ...

Elsevier2016

Glycogen metabolism and the homeostatic regulation of sleep

Pierre Magistretti, Igor Allaman, Jean-Marie Petit, Sophie Burlet Godinot

In 1995 Benington and Heller formulated an energy hypothesis of sleep centered on a key role of glycogen. It was postulated that a major function of sleep is to replenish glycogen stores in the brain that have been depleted during wakefulness which is asso ...

Springer Verlag2015

Deciphering the astrocyte-neuron metabolic cooperation in vivo

Manuel Raphael Zenger

Brain energy consumption is high and a very tightly regulated energy metabolism ensures correct performance. Neurons, the cells responsible for transmitting information via synapses, interact very closely with star shaped glial cells called astrocytes. Ast ...

EPFL2015

Imaging liver and brain glycogen metabolism at the nanometer scale

Anders Meibom, Graham Knott, Arnaud Comment, Stéphane Laurent Escrig, Yuhei Takado, Mojgan Masoodi

In mammals, glycogen synthesis and degradation are dynamic processes regulating blood and cerebral glucose-levels within a well-defined physiological range. Despite the essential role of glycogen in hepatic and cerebral metabolism, its spatiotemporal distr ...

Elsevier Science Bv2015

Disrupting astrocyte-neuron lactate transfer persistently reduces conditioned responses to cocaine

Pierre Magistretti, Jean-Luc Martin, Benjamin Boutrel, Benjamin Boury-Jamot, Anthony Carrard

A central problem in the treatment of drug addiction is the high risk of relapse often precipitated by drug-associated cues. The transfer of glycogen-derived lactate from astrocytes to neurons is required for long-term memory. Whereas blockade of drug memo ...

Nature Publishing Group2015

Assessing dystrophies and other muscle diseases at the nanometer scale by atomic force microscopy

Giovanni Dietler, Harald Brune, Andrzej Kulik, Nicolas Mermod, Guillaume Witz, Stefania Puttini

Aim: Atomic force microscopy nanoindentation of myofibers was used to assess and quantitatively diagnose muscular dystrophies from human patients. Materials & methods: Myofibers were probed from fresh or frozen muscle biopsies from human dystrophic patient ...

Future Medicine2014

Hepatic glucose sensing and integrative pathways in the liver

Kristina Schoonjans, Maaike Hélène Oosterveer

The hepatic glucose-sensing system is a functional network of enzymes and transcription factors that is critical for the maintenance of energy homeostasis and systemic glycemia. Here we review the recent literature on its components and metabolic actions. ...

Springer Basel Ag2014

Significant Molecular and Systemic Adaptations after Repeated Sprint Training in Hypoxia

Jean-Marc Vesin, Grégoire Millet

While intermittent hypoxic training (IHT) has been reported to evoke cellular responses via hypoxia inducible factors (HIFs) but without substantial performance benefits in endurance athletes, we hypothesized that repeated sprint training in hypoxia could ...

Public Library of Science2013

A comparison of in vivo (13) C MR brain glycogen quantification at 9.4 and 14.1 T

Rolf Gruetter, Yves Pilloud, Ruud van Heeswijk

The high molecular weight and low concentration of brain glycogen render its noninvasive quantification challenging. Therefore, the precision increase of the quantification by localized (13) C MR at 9.4 to 14.1 T was investigated. Signal-to-noise ratio inc ...

Wiley2012

Multidisciplinary Analysis of the Metabolic Shift to Lactate Consumption in CHO Cell Culture

Francesca Zagari

Mammalian cells represent the most widely used host system for the industrial production of recombinant therapeutic proteins. In order to increase productivity, chemically defined media are often used and optimized to provide the cells with the necessary n ...

EPFL2012