Bernard Schneider

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Publications associées (114)

Recovery of walking after paralysis by regenerating characterized neurons to their natural target region

Grégoire Courtine, Bernard Schneider, Jocelyne Bloch, Quentin Barraud, Jordan Squair, Mark Andrew Anderson, Katia Galan, Nicholas David James, Matthieu Pierre Gautier, Laetitia Danielle Philippine Baud

Axon regeneration can be induced across anatomically complete spinal cord injury (SCI), but robust functional restoration has been elusive. Whether restoring neurological functions requires directed regeneration of axons from specific neuronal subpopulatio ...

American Association for the Advancement of Science2023

Stable isotope labeling and ultra-high-resolution NanoSIMS imaging reveal alpha-synuclein-induced changes in neuronal metabolism in vivo

Anders Meibom, Bernard Schneider, Graham Knott, Stéphane Laurent Escrig, Louise Helene Soegaard Jensen, Bohumil Maco, Sofia Carmen Suzanne Spataro

In Parkinson's disease, pathogenic factors such as the intraneuronal accumulation of the protein alpha-synuclein affect key metabolic processes. New approaches are required to understand how metabolic dysregulations cause degeneration of vulnerable subtype ...

2023

AAV9-mediated SMN gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in Smn(2B/-) spinal muscular atrophy mice

Bernard Schneider

Structural, functional and molecular cardiac defects have been reported in spinal muscular atrophy (SMA) patients and mouse models. Previous quantitative proteomics analyses demonstrated widespread molecular defects in the severe Taiwanese SMA mouse model. ...

Oxford University Press2023

Differential effect of Fas activation on spinal muscular atrophy motoneuron death and induction of axonal growth

Bernard Schneider

Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) are the most common motoneuron diseases affecting adults and infants, respectively. ALS and SMA are both characterized by the selective degeneration of motoneurons. Although different in ...

2023

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

Dopamine and Methamphetamine Differentially Affect Electron Transport Chain Complexes and Parkin in Rat Striatum: New Insight into Methamphetamine Neurotoxicity

Bernard Schneider, Akhil Sharma

Methamphetamine (METH) is a highly abused psychostimulant that is neurotoxic to dopaminergic (DAergic) nerve terminals in the striatum and increases the risk of developing Parkinson's disease (PD). In vivo, METH-mediated DA release, followed by DA-mediated ...

MDPI2022

Gene therapy against amyotrophic lateral sclerosis: a bicistronic AAV vector for RNAi against mutated SOD1

Philippe Colin, Bernard Schneider, Pamela Solange Valdés Undurraga, Aline Aebi, Jean Philippe Gaudry

MARY ANN LIEBERT, INC2022

Central and peripheral delivered AAV9-SMN are both efficient but target different pathomechanisms in a mouse model of spinal muscular atrophy

Bernard Schneider

Spinal muscular atrophy (SMA) is a neuromuscular disease caused by loss of the SMN1 gene and low SMN protein levels. Although lower motor neurons are a primary target, there is evidence that peripheral organ defects contribute to SMA. Current SMA gene ther ...

2022

SMN Depleted Mice Offer a Robust and Rapid Onset Model of Nonalcoholic Fatty Liver Disease

Bernard Schneider

BACKGROUND & AIMS: Nonalcoholic fatty liver disease (NAFLD) is considered a health epidemic with potential devastating effects on the patients and the healthcare systems. Current preclinical models of NAFLD are invariably imperfect and generally take a lon ...

2021

CSP alpha reduces aggregates and rescues striatal dopamine release in alpha-synuclein transgenic mice

Bernard Schneider

alpha-Synuclein aggregation at the synapse is an early event in Parkinson's disease and is associated with impaired striatal synaptic function and dopaminergic neuronal death. The cysteine string protein (CSP alpha) and alpha-synuclein have partially overl ...

2021

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