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, 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

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

Bernard Schneider, Roman Chrast, Catherine Maclachlan, 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 ...

NATL ACAD SCIENCES2019

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

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

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 Univ Press2017

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

Collapsin response médiator protein 4a(CRMP 4a) is upregulated in motoneurons of mutant SOD1 mice and can trigger motoneuron death

Patrick Aebischer, Nathalie Marie Géraldine Bernard

Embryonic motoneurons from mutant SOD1 (mSOD1) mouse models of amyotrophic lateral sclerosis (ALS), but not wild-type motoneurons, can be triggered to die by exposure to nitric oxide (NO), leading to activation of a motoneuron-specific signaling pathway do ...

2010

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