Publications associées à Sclérose latérale amyotrophique

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

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, Soumya Banerjee, Greta Limoni, Samuel William Vernon, Wei Jiao

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

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, Antonella Romano

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, Nathalie Marie Géraldine Bernard, Cylia Cloé Rochat

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