A5004386868- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Nerve injury and regeneration
- Neuroinflammation and Neurodegeneration Mechanisms
- Muscle Physiology and Disorders
- Prion Diseases and Protein Misfolding
- Parkinson's Disease Mechanisms and Treatments
- Cell death mechanisms and regulation
- Cholinesterase and Neurodegenerative Diseases
- Cellular Mechanics and Interactions
- Neuroscience and Neuropharmacology Research
- Neurological diseases and metabolism
- Genetic Neurodegenerative Diseases
- Ion channel regulation and function
- Endoplasmic Reticulum Stress and Disease
- RNA Interference and Gene Delivery
- RNA Research and Splicing
- Cellular transport and secretion
- Neuroscience and Neural Engineering
- Signaling Pathways in Disease
- Polyomavirus and related diseases
- Cholesterol and Lipid Metabolism
- Fractal and DNA sequence analysis
- Peroxisome Proliferator-Activated Receptors
- Cancer-related gene regulation
Université de Montpellier
2014-2025
Institute for Neurosciences of Montpellier
2016-2025
Inserm
2016-2025
Centre Hospitalier Universitaire de Montpellier
2024
École Polytechnique Fédérale de Lausanne
2008-2024
Hôpital Saint Eloi
2013-2024
Centre National de la Recherche Scientifique
2000-2024
Trinity College Dublin
2024
Hôpital Gui de Chauliac
2024
St. James's Hospital
2024
About 50% of spinal motoneurons undergo programmed cell death (PCD) after target contact, but little is known about how this process initiated. Embryonic coexpress the receptor Fas and its ligand FasL at stage which PCD to begin. In absence trophic factors, many die in culture within 2 d. Most (75%) these were saved by Fas-Fc body, blocks interactions between FasL, or caspase-8 inhibitor tetrapeptide IETD. Therefore, activation endogenous underlies induced deprivation. presence neurotrophic...
Significance CD8 + T lymphocytes, which are typically devoted to eliminate malignant and infected cells, have been described in the central nervous system (CNS) of patients mice with amyotrophic lateral sclerosis (ALS). However, their role ALS pathogenesis has yet be unraveled. Here, we show that ablation cells increased number surviving motoneurons. expressing ALS-causing superoxide dismutase-1 mutant protein recognize selectively kill motoneurons vitro. To exert cytotoxic function,...
Amyotrophic lateral sclerosis (ALS) is predominantly associated with motor cortex, corticospinal tract (CST), brainstem, and spinal cord degeneration, cerebellar involvement much less well characterized. However, some of the cardinal clinical features ALS, such as dysarthria, dysphagia, gait impairment, falls, impaired dexterity, are believed to be exacerbated by coexisting pathology. Cerebellar pathology may also contribute cognitive, behavioral, pseudobulbar manifestations. Our objective...
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder arising from the selective death of motor neurons. Approximately 20% familial ALS (fALS) cases are caused by toxic gain-of-function mutations in superoxide dismutase 1 (SOD1) gene. We as well others have provided proof-of-principle for use RNA interference (RNAi) against mutant SOD1 potential therapy fALS. With aim maximizing delivery these silencing instructions, we explored efficacy intravenous recombinant...
Cellular responses to protein misfolding are thought play key roles in triggering neurodegeneration. In the mutant superoxide dismutase (mSOD1) model of amyotrophic lateral sclerosis (ALS), subsets motoneurons selectively vulnerable degeneration. Fast fatigable activate an endoplasmic reticulum (ER) stress response that drives their early degeneration while a subset mSOD1 show exacerbated sensitivity activation motoneuron-specific Fas/NO pathway. However, links between two mechanisms and...
The selective degeneration of motoneuron that typifies amyotrophic lateral sclerosis (ALS) implicates non‐cell‐autonomous effects astrocytes. However, mechanisms underlying astrocyte‐mediated neurotoxicity remain largely unknown. According to the determinant role astrocyte metabolism in supporting neuronal function, we propose explore metabolic status astrocytes exposed ALS‐associated conditions. We found a significant dysregulation including purine, pyrimidine, lysine, and...
The reasons for the cellular specificity and slow progression of motoneuron diseases such as ALS are still poorly understood. We previously described a motoneuron-specific cell death pathway downstream Fas receptor, in which synthesis nitric oxide (NO) is an obligate step. Motoneurons from model mice expressing mutant SOD1 showed increased susceptibility to exogenous NO compared with controls. Here, we report signaling mechanism whereby leads mutant, but not control, motoneurons....
Background: auto-antibodies against the potassium channel inward rectifying 4.1 (Kir4.1) have previously been identified in 46% of patients with multiple sclerosis (MS). Objectives: to confirm these findings. Methods: we evaluated presence anti-Kir4.1 antibodies by enzyme-linked immunosorbent assay (ELISA) and immunofluorescence 268 MS patients, 46 other neurological diseases (OND) 45 healthy controls. Results: were found 7.5% 4.3% OND 4.4% Immunofluorescence analysis did not identify any...
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting motor neurons. Recently, genome-wide association studies identified KIF5A as new ALS-causing gene. encodes protein of the kinesin-1 family, allowing anterograde transport cargos along microtubule rails in In ALS patients, mutations gene induce exon 27 skipping, resulting mutated with C-terminal region (KIF5A Δ27). To understand how Δ27 underpins disease, we developed an ALS-associated Drosophila model. When...
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 nitric oxide (NO), leading activation a motoneuron-specific signaling pathway downstream the death receptor Fas/CD95. To identify effectors mSOD1-dependent cell death, we performed proteomic analysis. Treatment cultured mSOD1 with NO led 2.5-fold increase in levels collapsin response mediator protein 4a (CRMP4a) . In vivo ,...
A dominant mutation in the gene coding for vesicle-associated membrane protein-associated protein B (VAPB) was associated with amyotrophic lateral sclerosis, a fatal paralytic disorder characterized by selective loss of motoneurons brain and spinal cord. Adeno-associated viral vectors that we show to transduce up 90% vitro were used model VAPB-associated neurodegenerative process. We observed viral-mediated over-expression both wild-type mutated form human VAPB selectively induces death...
Glutamate-induced excitotoxicity is a major contributor to motor neuron degeneration in the pathogenesis of amyotrophic lateral sclerosis (ALS). The spinal cord × Neuroblastoma hybrid cell line (NSC-34) often used as bona fide cellular model investigate physiopathological mechanisms ALS. However, physiological response NSC-34 glutamate remains insufficiently described. In this study, we evaluated relevance differentiated (NSC-34D) an vitro for studies. NSC-34D showed morphological and...
Amyotrophic lateral sclerosis (ALS) is a fatal and incurable paralytic disorder caused by the progressive death of upper lower motoneurons. Although numerous strategies have been developed to slow disease progression improve life quality, date only few therapeutic treatments are available with still unsatisfactory benefits. The secretome dental pulp stem cells (DPSCs) contains neurotrophic factors that could promote motoneuron survival. Accordingly, DPSCs confer neuroprotective benefits