Concept

Sclérose latérale amyotrophique

Publications associées (74)

Genetic Mouse Models of Neurodegenerative Diseases

Darren Moore, Alzbeta Trancikova, David Ramonet Jimenez

Neurodegenerative diseases are generally characterized by the selective degeneration of particular neuronal populations and the accumulation of abnormal or aggregated proteins within, but occasionally external to, neurons in affected brain regions. These d ...

2011

Rab1A Over-Expression Prevents Golgi Apparatus Fragmentation and Partially Corrects Motor Deficits in an Alpha-Synuclein Based Rat Model of Parkinson’s Disease

Patrick Aebischer, Bernard Schneider, Jean-Charles Bensadoun, Philippe Guillaume Coune

Although the overabundance of human alpha-synuclein in nigral dopaminergic neurons is considered to play a pathogenic role in Parkinson's disease (PD), it remains unclear how alpha-synuclein leads to neuronal degeneration and motor symptoms. Here, we explo ...

Ios Press2011

NR2B subunit blockade does not affect motor symptoms induced by 3-nitropropionic acid

Martina Fantin

Broad-spectrum N-methyl D-aspartate (NMDA) antagonists, although proposed in therapies for several pathologies including Huntington's disease (HD), can produce dramatic side-effects. Thus, the therapeutic potential of subunit selective NMDA receptor antago ...

2011

Adeno-Associated Virus-Mediated Gene Delivery for the Treatment of Amyotrophic Lateral Sclerosis and other Motor Neuron Disorders

Christopher Towne

The delivery of molecules and genes to the central nervous system (CNS) poses a major challenge for the treatment of neurodegenerative diseases. CNS disorders require long-term intervention and the presence of the blood-brain barrier (BBB) restricts the pe ...

EPFL2010

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

Upper Limb Spasticity Reduction Following Active Training: A Robot-Mediated Study In Patients With Chronic Hemiparesis

Silvestro Micera

Objective: To compare the effects on spasticity of 2 robot-mediated therapies in patients with chronic hemiparesis. ...

2010

Processing of the synaptic cell adhesion molecule neurexin-3beta by Alzheimer disease alpha- and gamma-secretases

Patrick Fraering, Claude André Schweizer, Saoussen Ben Halima, Nathalie Bot

Neurexins (NRXNs) are synaptic cell adhesion molecules having essential roles in the assembly and maturation of synapses into fully functional units. Immunocytochemical and electrophysiological studies have shown that specific binding across the synaptic c ...

American Society for Biochemistry and Molecular Biology2010

Lentiviral and Adeno-Associated Vector-Based Therapy for Motor Neuron Disease Through RNAi

Patrick Aebischer, Christopher Towne

RNAi holds promise for neurodegenerative disorders caused by gain-of-function mutations. We and others have demonstrated proof-of-principle for viral-mediated RNAi in a mouse model of motor neuron disease. Lentivirus and adeno-associated virus have been us ...

2009

Rapid Functional Maturation of Nascent Dendritic Spines

Graham Knott

Spine growth and retraction with synapse formation and elimination plays an important role in shaping brain circuits during development and in the adult brain, yet the temporal relationship between spine morphogenesis and the formation of functional synaps ...

2009

Spinal Cord Stimulation Restores Locomotion in Animal Models of Parkinson's Disease

Miguel Nicolelis

Dopamine replacement therapy is useful for treating motor symptoms in the early phase of Parkinson's disease, but it is less effective in the long term. Electrical deep-brain stimulation is a valuable complement to pharmacological treatment but involves a ...

2009

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