Publications de Nathalie Marie Géraldine Bernard

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

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

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

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

Endoplasmic reticulum and mitochondria in diseases of motor and sensory neurons: a broken relationship?

Bernard Schneider, Roman Chrast, Nathalie Marie Géraldine Bernard

Nature Publishing Group2018

PFN2 and GAMT as common molecular determinants of axonal Charcot-Marie-Tooth disease

Bernard Schneider, Manisha Juneja, Roman Chrast, Nathalie Marie Géraldine Bernard

2018

Parkin functionally interacts with PGC-1 proportional to to preserve mitochondria and protect dopaminergic neurons

Patrick Aebischer, Bernard Schneider, Graham Knott, Johan Auwerx, Darren Moore, Norman Lionel Moullan, Lu Zheng, Nathalie Marie Géraldine Bernard, Davide D'Amico

To understand the cause of Parkinson's disease (PD), it is important to determine the functional interactions between factors linked to the disease. Parkin is associated with autosomal recessive early-onset PD, and controls the transcription of PGC-1a, a m ...

Oxford University Press2017

Use of a Bicistronic Vector to Silence SOD1 in Motoneurons and Astrocytes for the Treatment of Familial Amyotrophic Lateral Sclerosis

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

Nature Publishing Group2016

Characterization Of Motor And Sensory Neuronal Dysfunction In Both In Vitro And In Vivo Models Of Cmt2A Pathology

Bernard Schneider, Cylia Cloé Rochat, Roman Chrast, Nathalie Marie Géraldine Bernard

Wiley-Blackwell2016

Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration

Roman Chrast, Nathalie Marie Géraldine Bernard

Mutations in SIGMAR1, which encodes the Sigma 1 receptor, cause a familial form of amyotrophic lateral sclerosis, but the underlying molecular mechanisms are unclear. Bernard-Marissal et al. reveal that disruption of Sigma 1 receptor function disturbs endo ...

Oxford University Press2015

Reply: Is SIGMAR1 a confirmed FTD/MND gene?

Roman Chrast, Nathalie Marie Géraldine Bernard

Oxford University Press2015