Bernard Schneider

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Publications associées (114)

Altered interplay between endoplasmic reticulum and mitochondria in Charcot-Marie-Tooth type 2A neuropathy

Bernard Schneider, Mary-Claude Croisier-Coeytaux, Catherine Maclachlan, Roman Chrast, Nathalie Marie Géraldine Bernard, Cylia Cloé Rochat, Omar El Mansour, Manisha Juneja

Mutations in the MFN2 gene encoding Mitofusin 2 lead to the development of Charcot-Marie-Tooth type 2A (CMT2A), a dominant axonal form of peripheral neuropathy. Mitofusin 2 is localized at both the outer membrane of mitochondria and the endoplasmic reticul ...

National Academy of Sciences2019

The RNA-Binding Protein PUM2 Impairs Mitochondrial Dynamics and Mitophagy During Aging

Johan Auwerx, Kristina Schoonjans, Bernard Schneider, Graham Knott, Davide D'Amico, Vera Monica Lemos Da Silva, Mario Romani, Vincenzo Sorrentino, Adrienne Joëlle Laurence Mottis, Hao Li

Little information is available about how post-transcriptional mechanisms regulate the aging process. Here, we show that the RNA-binding protein Pumilio2 (PUM2), which is a translation repressor, is induced upon aging and acts as a negative regulator of li ...

CELL PRESS2019

Allele-specific gene disruption through discrimination of a single base change by S. aureus Cas9-KKH prevents progressive hearing loss after AAV-mediated gene delivery

Bernard Schneider, Paola Andrea Solanes Vega, Sofia Carmen Suzanne Spataro

MARY ANN LIEBERT, INC2019

Scalable Production of AAV Vectors in Orbitally Shaken HEK293 Cells

Florian Maria Wurm, Bernard Schneider, Vivianne Padrun, Nicole Déglon, Daniel Roman Blessing, Gabriel Vachey

Adeno-associated virus (AAV) vectors are currently among the most commonly applied for in vivo gene therapy approaches. The evaluation of vectors during clinical development requires the production of considerable amounts of highly pure and potent vectors. ...

2019

Delivery of CRISPR/Cas9 using AAV-PHP.B in the inner ear leads to allele-specific inactivation of the mutated Tmc1 allele and protects auditory function in Beethoven mice

Bernard Schneider, Paola Andrea Solanes Vega, Sofia Carmen Suzanne Spataro

Hearing Loss (HL) is the most prevalent sensorineural disorder, affecting 360 million people worldwide. As genetic causes lead to 50% of pre-lingual deafness, gene therapy is considered as a potential therapeutic strategy. Mutations in the TMC1 gene are li ...

MARY ANN LIEBERT, INC2019

Glutaredoxin1 Diminishes Amyloid Beta-Mediated Oxidation of F-Actin and Reverses Cognitive Deficits in an Alzheimer's Disease Mouse Model

Bernard Schneider

Aims: Reactive oxygen species (ROS) generated during Alzheimer's disease (AD) pathogenesis through multiple sources are implicated in synaptic pathology observed in the disease. We have previously shown F-actin disassembly in dendritic spines in early AD ( ...

2019

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

Rolf Gruetter, Patrick Aebischer, Bernard Schneider, Hongxia Lei, 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

Compositions and methods for delivering nucleic acids to cochlear and vestibular cells

Bernard Schneider, Paola Andrea Solanes Vega

Provided herein are materials and methods for efficiently delivering nucleic acids to cochlear and vestibular cells, and methods of treating sensory transduction disorders associated with a genetic defect. ...

2019

Pathogenic commonalities between spinal muscular atrophy and amyotrophic lateral sclerosis: Converging roads to therapeutic development

Bernard Schneider

Spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS) are the two most common motoneuron disorders, which share typical pathological hallmarks while remaining genetically distinct. Indeed, SMA is caused by deletions or mutations in the surv ...

ELSEVIER SCIENCE BV2018

Cortico–reticulo–spinal circuit reorganization enables functional recovery after severe spinal cord contusion

Grégoire Courtine, Bernard Schneider, Quentin Barraud, Léonie Asboth, Mark Andrew Anderson, Lucia Florinda Friedli Wittler, Laetitia Danielle Philippine Baud, Polina Shkorbatova, Janine Beauparlant, Galyna Pidpruzhnykova

Severe spinal cord contusions interrupt nearly all brain projections to lumbar circuits producing leg movement. Failure of these projections to reorganize leads to permanent paralysis. Here we modeled these injuries in rodents. A severe contusion abolished ...

2018

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