Publications associées à Systemic AAV6 delivery mediating RNA interference against SOD1: neuromuscular transduction does not alter disease progression in fALS mice

Publications associées (33)

A Spatiotemporal Analysis of Brain Dynamics, a New Feature for Sleep and Seizure State Discrimination

The human brain is a complex and dynamic physiological system that can be considered as a network of reciprocally interconnected systems localized in different brain regions. To fully characterize the brain's complex dynamics, it is essential to investigat ...

IEEE2025

Capturing behavioral and neural variability in multi-area spiking neuronal network models

Christos Sourmpis

How does the brain generate behavioral responses from sensory stimuli? This sensor-to-motor transformation is a fundamental question in systems neuroscience. While recent experiments have highlighted the complexity of neural representations across the cere ...

EPFL2025

Trio preserves motor synapses and prolongs motor ability during aging

Evelyne Ruchti, Brian Donal McCabe, Wei Jiao, Soumya Banerjee, Greta Limoni, Samuel William Vernon, Marine Van Campenhoudt

The decline of motor ability is a hallmark feature of aging and is accompanied by degeneration of motor synaptic terminals. Consistent with this, Drosophila motor synapses undergo characteristic age -dependent structural fragmentation co -incident with dim ...

Cell Press2024

Cyclo His-Pro Attenuates Muscle Degeneration in Murine Myopathy Models

Johan Auwerx, Xiaoxu Li, Tanes Imamura de Lima, Alessia De Masi, Keno Strotjohann

Among the inherited myopathies, a group of muscular disorders characterized by structural and metabolic impairments in skeletal muscle, Duchenne muscular dystrophy (DMD) stands out for its devastating progression. DMD pathogenesis is driven by the progress ...

Wiley2024

Evaluation of antigravitational support levels provided by a passive upper-limb occupational exoskeleton in repetitive arm movements

Giulia Ramella

Upper-limb occupational exoskeletons to support the workers' upper arms are typically designed to provide antigravitational support. Although typical work activities require workers to perform static and dynamic actions, the majority of the studies in lite ...

Elsevier Sci Ltd2024

Targeting TDP-43 Proteinopathies in Neurodegeneration by its Native-State Stabilization

Lixin Yang

Preventing the misfolding or aggregation of TDP-43 is the most actively pursued disease-modifying strategy to treat amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. In our work, we provide proof of concept that native state stabili ...

EPFL2024

Uncovering interactions between Descending Neurons as a functional principle of behavioural control

Jonas Friedrich Braun

Animals, including humans, exhibit a remarkable variety of complex behaviours. How the nervous system controls all these behaviours ranging from simple, stereotyped movements to flexible, adaptive actions is a central questions of neuroscience. One of the ...

EPFL2024

Multimodal transcriptomic atlases of mouse spinal cord injury

Matthieu Pierre Gautier

A spinal cord injury (SCI) triggers a cascade of molecular and cellular responses involving inflammatory cell infiltration and cytokine release, apoptosis, demyelination, excitotoxicity, ischemia, and the formation of a fibrotic scar surrounded by an astro ...

EPFL2024

Accessible and highly observable gastrointestinal organoid model systems for studying host-pathogen interactions

Moritz Hofer

Traditional cell cultures have long been fundamental to biological research, offering an alternative to animal models burdened by ethical constraints and procedural intricacies, often lacking relevance to human physiology and disease. Moreover, their inabi ...

EPFL2024

Effects of multidisciplinary inpatient rehabilitation on everyday life physical activity and gait in patients with multiple sclerosis

Kamiar Aminian, Anisoara Ionescu, Gaëlle Prigent

Background Multiple sclerosis is a progressive neurological disease that affects the central nervous system, resulting in various symptoms. Among these, impaired mobility and fatigue stand out as the most prevalent. The progressive worsening of symptoms ad ...

BioMed Central2024

Loss of Motor Cortical Inputs to the Red Nucleus after CNS Disorders in Nonhuman Primates

Simon Borgognon

The premotor (PM) and primary motor (M1) cortical areas broadcast voluntary motor commands through multiple neuronal pathways, including the corticorubral projection that reaches the red nucleus (RN). However, the respective contribution of M1 and PM to co ...

SOC NEUROSCIENCE2023

Digital reconstruction of the mammalian spinal cord: from anatomical reference volume to cell type atlas of the mouse spinal cord

Ihor Kuras

The spinal cord is an elongated nervous structure that together with the brain forms the central nervous system. It relays sensory and motor information between the brain and the body, thus controlling most somatic and autonomic body functions. In recent y ...

EPFL2023

Brain-controlled spinal cord stimulation to restore mobility after spinal cord injury

Carmina Andrea Galvez Solano

Spinal Cord Injury (SCI) affects almost 500,000 people every year, causing complete paralysis of both legs in severe cases, with no current treatment perspective. However, new neuroengineering technologies, such as the Brain Spine Interface (BSI), have eme ...

EPFL2023

Neurotechnologies to restore hand functions

Silvestro Micera, Solaiman Shokur

Reaching and manipulating objects are crucial tasks that allow proactive interaction with our surroundings. However, these functions are lost after neurological disorders or traumatic events that cause hand paralysis. Neuroprosthetic technologies are medic ...

SPRINGERNATURE2023

Targeted regeneration of spinal cord neurons across a complete injury restores walking

Marco Milano

Spinal cord injury (SCI) interrupts axonal connections between the brain and the spinal cord, and is characterized by a spectrum of sensorimotor and autonomic impairments. While spontaneous recovery is limited, recent studies have shown that functional imp ...

EPFL2023

Understanding motor control and impairment: An upper-limb model for muscular assessment

Tristan Jean Robin Barjavel

The human upper limb is a complex musculoskeletal system that can still perform various tasks with impressive efficacy thanks to the ability of the central nervous system to control and modulate the activation of more than 40 muscles. Stroke is a leading ...

EPFL2023

SiliFish: A desktop application to model swimming behavior in developing zebrafish (Danio rerio)

Yann Roussel

SiliFish is an open-source desktop application tomodel and study zebrafish swimming. Here, we explain how to define the general parameters of the model, define cell populations, place them within the spinal cord, and define their projections. We explain ho ...

Elsevier2023

Novel Design and Implementation of a Neuromuscular Controller on a Hip Exoskeleton for Partial Gait Assistance

Auke Ijspeert, Mohamed Bouri, Andrea Di Russo, Ali Reza Manzoori, Sara Messara

Exoskeletons intended for partial assistance of walking should be able to follow the gait pattern of their users, via online adaptive control strategies rather than imposing predefined kinetic or kinematic profiles. NeuroMuscular Controllers (NMCs) are ada ...

2023

Inhibiting de novo ceramide synthesis restores mitochondrial and protein homeostasis in muscle aging

Dohyun Park, Johan Auwerx, Xiaoxu Li, Hao Li, Jean-David Horacio Morel, Tanes Imamura de Lima, Ludger Jan Elzuë Goeminne, Pirkka-Pekka Untamo Laurila, Martin Rainer Wohlwend, Mario Romani, Sandra Rodriguez Lopez, Changmyung Oh

Disruption of mitochondrial function and protein homeostasis plays a central role in aging. However, how these processes interact and what governs their failure in aging remain poorly understood. Here, we showed that cer- amide biosynthesis controls the de ...

AMER ASSOC ADVANCEMENT SCIENCE2023

Fast-Spiking Interneurons of the Premotor Cortex Contribute to Initiation and Execution of Spontaneous Actions

Silvestro Micera

Planning and execution of voluntary movement depend on the contribution of distinct classes of neurons in primary motor and premotor areas. However, timing and pattern of activation of GABAergic cells during specific motor behaviors remain only partly unde ...

SOC NEUROSCIENCE2023