Publications associées à Response of aged parkinsonian monkeys to in vivo gene transfer of GDNF

Engineering synthetic adhesins for biophysical investigation and rewiring of host-microbe interactions

Host attachment is often a critical step in the onset of pathogenesis. To attach to host cells, bacteria have evolved a range of adhesins that bind to specific receptors. Some of these adhesins have been thoroughly characterized using biochemical technique ...

EPFL2022

The role of medial amygdala inhibitory neurons in regulating social behavior

Aiste Baleisyte

Aggression is an evolutionary conserved social behavior that is necessary for an animalÂŽs survival. The neural substrates coordinating aggression are known to exist in the amygdala and the hypothalamus. While the medial amygdala (MeA) and the ventrolatera ...

EPFL2021

Gene replacement therapy provides benefit in an adult mouse model of Leigh syndrome

Johan Auwerx

Mutations in nuclear-encoded mitochondrial genes are responsible for a broad spectrum of disorders among which Leigh syndrome is the most common in infancy. No effective therapies are available for this severe disease mainly because of the limited capabili ...

2020

Glutaredoxin 1 Downregulation in the Substantia Nigra Leads to Dopaminergic Degeneration in Mice

Bernard Schneider

Background Parkinson's disease (PD) is characterized by a severe loss of the dopaminergic (DA) neurons in the substantia nigra pars compacta (SNpc). Perturbation of protein thiol redox homeostasis has been shown to play a role in the dysregulation of cell ...

2020

Dopaminergic neurodegeneration induced by Parkinson's disease-linked G2019S LRRK2 is dependent on kinase and GTPase activity

Darren Moore, Elpida Tsika, Xi Chen

Mutations in leucine-rich repeat kinase 2 (LRRK2) are the most common cause of late-onset, autosomal-dominant familial Parkinson's disease (PD). LRRK2 functions as both a kinase and GTPase, and PD-linked mutations are known to influence both enzymatic acti ...

National Academy of Sciences2020

MRI-based brain volumetry and retinal optical coherence tomography as the biomarkers of outcome in acute methanol poisoning

Bénédicte Marie Maréchal, Tobias Kober

Background: Basal ganglia lesions are typical findings on magnetic resonance imaging (MRI) of the brain in survivors of acute methanol poisoning. However, no data are available on the association between the magnitude of damaged brain regions, serum concen ...

ELSEVIER2020

Nano-imaging trace elements at organelle levels in substantia nigra overexpressing alpha-synuclein to model Parkinson's disease

Philippe Colin, Bernard Schneider, Graham Knott

Sub-cellular trace element quantifications of nano-heterogeneities in brain tissues offer unprecedented ways to explore at elemental level the interplay between cellular compartments in neurodegenerative pathologies. We designed a quasi-correlative method ...

NATURE PUBLISHING GROUP2020

Pronounced α-synuclein pathology in a seeding-based mouse model is not sufficient to induce mitochondrial respiration deficits in the striatum and amygdala

Maria del Carmen Sandi Perez, Hilal Lashuel, Jean-Christophe Copin, Johannes Burtscher

Increasing evidence suggests that crosstalk between α-synuclein pathology formation and mitochondrial dysfunction plays a central role in the pathogenesis of Parkinson’s disease and related synucleinopathies. While mitochondrial dysfunction is a well-studi ...

2020

Efficient viral transduction in mouse inner ear hair cells with utricle injection and AAV9-PHP.B

Bernard Schneider, Paola Andrea Solanes Vega

Viral delivery of exogenous coding sequences into the inner ear has the potential for therapeutic benefit for patients suffering genetic or acquired hearing loss. To devise improved strategies for viral delivery, we investigated two injection techniques, r ...

2020

Metabolic and hemodynamic changes in the activated human brain and cerebellar organisation measured by MRS/MRl at 7 Tesla

Yohan Boillat

Although less often investigated than the positive blood-oxygen-level-dependent (BOLD) signal, a negative BOLD signal has also been observed under certain conditions, which could have a neuronal origin. To further investigate the negative BOLD signal from ...

EPFL2020

Required growth facilitators propel axon regeneration across complete spinal cord injury

Sabry Leonardo Barlatey, Grégoire Courtine, Cheng Wang, Mark Andrew Anderson, Michael V. Sofroniew, Zhigang He, Nicholas David James

Transected axons fail to regrow across anatomically complete spinal cord injuries (SCI) in adults. Diverse molecules can partially facilitate or attenuate axon growth during development or after injury1,2,3, but efficient reversal of this regrowth failure ...

2018

Data-driven reconstruction of a point neuron mouse brain

Csaba Erö

In this work, we present a semi-automatic method to reconstruct a mouse whole-brain model at the point-neuron level by integrating a wide array of biological data. Our process has three parts: cell position and type assignment, connectivity mapping, and si ...

EPFL2018

Preferential Enhancement of Sensory and Motor Axon Regeneration by Combining Extracellular Matrix Components with Neurotrophic Factors

Silvestro Micera, Daniel Santos

After peripheral nerve injury, motor and sensory axons are able to regenerate but inaccuracy of target reinnervation leads to poor functional recovery. Extracellular matrix (ECM) components and neurotrophic factors (NTFs) exert their effect on different ne ...

2017

AAV based gene therapy for rare diseases : targeting astrocytes for functional recovery in Amyotrophic Lateral Sclerosis

Cylia Cloé Rochat

Of the seven thousand diseases that are described as rare, 80% of them have an identified genetic cause. With this in the mind, the development of technologies, such as gene therapy, to address the genetic factors involved in these pathologies, might be a ...

EPFL2017

The effect of the functional interaction between PGC-1α and Parkin on mitochondrial quality control in Parkinson's disease

Lu Zheng

Although several genetic factors have been directly associated to Parkinsonâ s disease (PD), no single pathway has emerged to explain the etiology of this neurodegenerative disease. To further understand the cause of Parkinsonâ s disease (PD), it is ther ...

EPFL2016

alpha-Synuclein-induced dopaminergic neurodegeneration in a rat model of Parkinson's disease occurs independent of ATP13A2 (PARK9)

Liliane Glauser, Bernard Schneider, Darren Moore, Alessandra Musso, Elpida Tsika, Aris Nikolaos Fiser, Guillaume Daniel

Mutations in the ATP13A2 (PARK9) gene cause early-onset, autosomal recessive Parkinson's disease (PD) and Kufor-Rakeb syndrome. ATP13A2 mRNA is spliced into three distinct isoforms encoding a P5-type ATPase involved in regulating heavy metal transport acro ...

Academic Press Inc Elsevier Science2015

Lack of additive role of ageing in nigrostriatal neurodegeneration triggered by alpha-synuclein overexpression

Grégoire Courtine, Isabel Laura Vollenweider, Laetitia Danielle Philippine Baud

Introduction: Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by the loss of dopaminergic neurons as well as the presence of proteinaceous inclusions named Lewy bodies. alpha synuclein (alpha-syn) is a major constituent o ...

2015

Astrocytes: New Targets for the Treatment of Neurodegenerative Diseases

Sylvain Eric Lengacher, Pierre Magistretti, Charles Léonard Finsterwald

The causes of neurodegenerative disorders are multiple, and for most of them a mechanistic understanding is still lacking. However, neurodegenerative diseases such as Alzheimer disease (AD), amyotrophic lateral sclerosis (ALS) and Parkinson disease (PD) al ...

Bentham Science Publishers2015

Inferior frontal oscillations reveal visuo-motor matching for actions and speech: evidence from human intracranial recordings

Olaf Blanke, Pär Halje, Silvio Ionta

The neural correspondence between the systems responsible for the execution and recognition of actions has been suggested both in humans and non-human primates. Apart from being a key region of this visuo-motor observation-execution matching (OEM) system, ...

Pergamon-Elsevier Science Ltd2015

PGC-1 alpha activity in nigral dopamine neurons determines vulnerability to alpha-synuclein

Bernard Schneider, Graham Knott, Wojciech Janusz Bobela, Lu Zheng

Introduction: Mitochondrial dysfunction and oxidative stress are critical factors in the pathogenesis of age-dependent neurodegenerative diseases. PGC-1 alpha, a master regulator of mitochondrial biogenesis and cellular antioxidant defense, has emerged as ...

2015