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Concept

Sclérose latérale amyotrophique

Publications associées (27)

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

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

Discovery of macrocyclic inhibitors of challenging protein-protein interactions

Edward Jeffrey Will

Macrocycles are a therapeutic modality of interest for drugging challenging disease targets due to their high binding affinity and membrane permeability, enabling the discovery of orally delivered and efficacious therapeutics. Recent methods have enabled t ...

EPFL2024

The role of the NF-kappaB pathway in Amyotrophic lateral sclerosis pathogenesis

Emma Charlotta Källstig

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative motor disorder, which results in death within a few years of diagnosis. While the cause of most cases of ALS is unknown, 10% of cases are familial (fALS), and associated with mutations in one of ov ...

EPFL2023

A digital bridge between brain and spinal cord restores walking after paralysis

Grégoire Courtine, Jocelyne Bloch

Spinal-cord injury interrupts communication between the brain and spinal cord, leading to paralysis. An implant that decodes the brain signals that control movements and drives electrical stimulation of the spinal cord re-establishes this communication, en ...

NATURE PORTFOLIO2023

Effective Inhibition of TDP-43 Aggregation by Native State Stabilization

Hilal Lashuel, Yllza Jasiqi, Lixin Yang

Preventing the misfolding or aggregation of transactive response DNA binding protein with 43 kDa (TDP-43) is the most actively pursued disease-modifying strategy to treat amyotrophic lateral sclerosis and other neurodegenerative diseases. In this work, we ...

Wiley-VCH Verlag GmbH2024

The progressive loss of brain network fingerprints in Amyotrophic Lateral Sclerosis predicts clinical impairment

Enrico Amico

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by functional connectivity alterations in both motor and extra-motor brain regions. Within the framework of network analysis, fingerprinting represents a reliable approach to ...

ELSEVIER SCI LTD2022

Astrocyte-targeting RNA interference against mutated superoxide dismutase 1 induces motoneuron plasticity and protects fast-fatigable motor units in a mouse model of amyotrophic lateral sclerosis

Patrick Aebischer, Bernard Schneider, Cylia Cloé Rochat, Nathalie Marie Géraldine Bernard

In amyotrophic lateral sclerosis (ALS) caused by SOD1 gene mutations, both cell-autonomous and noncell-autonomous mechanisms lead to the selective degeneration of motoneurons (MN). Here, we evaluate the therapeutic potential of gene therapy targeting mutat ...

WILEY2022

Cortical and subcortical changes in resting-state neuronal activity and connectivity in early symptomatic ALS and advanced frontotemporal dementia

Alessandra Griffa

The objective of this study was to examine if patterns of resting-state brain activity and functional connectivity in cortical and subcortical regions in patients with early symptomatic amyotrophic lateral sclerosis (ALS) resemble those of behavioural vari ...

ELSEVIER SCI LTD2022

The Links between ALS and NF-κB

Bernard Schneider, Brian Donal McCabe, Emma Charlotta Källstig

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease wherein motor neu-ron degeneration leads to muscle weakness, progressive paralysis, and death within 3–5 years ofdiagnosis. Currently, the cause of ALS is unknown but, as with several neuro ...

2021

The phase separation-dependent FUS interactome reveals nuclear and cytoplasmic function of liquid-liquid phase separation

Helen Lindsay

Liquid-liquid phase separation (LLPS) of proteins and RNAs has emerged as the driving force underlying the formation of membrane-less organelles. Such biomolecular condensates have various biological functions and have been linked to disease. The protein F ...

2021

Robot-induced hallucinations in Parkinson’s disease depend on altered sensorimotor processing in fronto-temporal network

Dimitri Nestor Alice Van De Ville, Olaf Blanke, Nathan Quentin Faivre, Giulio Rognini, Fosco Bernasconi, Michela Bassolino, Jevita Potheegadoo, Michel Akselrod, Eva Blondiaux, Giedre Stripeikyte, Matteo Franza

Hallucinations in Parkinson’s disease (PD) are disturbing and frequent non-motor symptoms and constitute a major risk factor for psychosis and dementia. We report a robotics-based approach applying conflicting sensorimotor stimulation, enabling the inducti ...

2021

On Modeling Glottal Source Information for Phonation Assessment in Parkinson's Disease

Mathew Magimai Doss, Julian David Fritsch

Parkinson's disease produces several motor symptoms, including different speech impairments that are known as hypokinetic dysarthria. Symptoms associated to dysarthria affect different dimensions of speech such as phonation, articulation, prosody, and inte ...

ISCA-INT SPEECH COMMUNICATION ASSOC2021

Smart Armband with Graphene Textile Electrodes for EMG-based Muscle Fatigue Monitoring

Ata Jedari Golparvar

We report the successful acquisition of surface electromyography (sEMG) signals from an intelligent armband and its application in localized muscle fatigue monitoring with a costume-designed, small-scale front-end readout circuitry. The correlation coeffic ...

IEEE2021

Mechanotaxis directs Pseudomonas aeruginosa twitching motility

Alexandre Louis André Persat, Lorenzo Anton-Louis Talà, Xavier Jean-Yves Pierrat, Jose Negrete Jr, Zaïnebe Dhirani, Marco Julian Kühn

The opportunistic pathogen Pseudomonas aeruginosa explores surfaces using twitching motility powered by retractile extracellu-lar filaments called type IV pili (T4P). Single cells twitch by sequen-tial T4P extension, attachment, and retraction. How single ...

National Academy of Sciences2021

Parkinson mice show functional and molecular changes in the gut long before motoric disease onset

Hilal Lashuel

Background: There is increasing evidence that Parkinson's disease (PD) might start in the gut, thus involving and compromising also the enteric nervous system (ENS). At the clinical onset of the disease the majority of dopaminergic neurons in the midbrain ...

2021

Evaluation of the NAD(+) biosynthetic pathway in ALS patients and effect of modulating NAD(+) levels in hSOD1-linked ALS mouse models

Johan Auwerx

Amyotrophic lateral sclerosis (ALS) is characterized by progressive degeneration of motor neurons. Astrocytes from diverse ALS models induce motor neuron death in co-culture. Enhancing NAD(+) availability, or increasing the expression of the NAD(+)-depende ...

2020

Lack of effect on ambulation of dalfampridine-ER (4-AP) treatment in adult SMA patients

Brian Donal McCabe

SMA is a genetically determined motor system disorder that results in muscle weakness, selective motor neuron death, muscle atrophy, and impaired functional mobility. In SMA model systems, long-term treatment with 4-aminopyridine (4-AP) has been shown to i ...

PERGAMON-ELSEVIER SCIENCE LTD2020

Evolution of the neurochemical profiles in the G93A-SOD1 mouse model of amyotrophic lateral sclerosis

Rolf Gruetter, Patrick Aebischer, Hongxia Lei, Bernard Schneider, Carole Poitry-Yamate, Elisabeth Anne Dirren

In vivo (1)H magnetic resonance spectroscopy ((1)H-MRS) investigations of amyotrophic lateral sclerosis (ALS) mouse brain may provide neurochemical profiles and alterations in association with ALS disease progression. We aimed to longitudinally follow neur ...

2019

FM19G11-Loaded Gold Nanoparticles Enhance the Proliferation and Self-Renewal of Ependymal Stem Progenitor Cells Derived from ALS Mice

Francesco Stellacci

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons. In ALS mice, neurodegeneration is associated with the proliferative restorative attempts of ependymal stem progenitor cells (epSPCs) that normally lie i ...

2019