Search Results - "Dieterlé, Stéphane"
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1
Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss
Published in The EMBO journal (17-05-2016)“…FUS is an RNA‐binding protein involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Cytoplasmic FUS‐containing aggregates are…”
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2
Evaluation of a 5-HT2B receptor agonist in a murine model of amyotrophic lateral sclerosis
Published in Scientific reports (08-12-2021)“…Degeneration of brainstem serotonin neurons has been demonstrated in ALS patients and mouse models and was found responsible for the development of spasticity…”
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3
FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis
Published in Nature neuroscience (01-11-2019)“…Neuromuscular junction (NMJ) disruption is an early pathogenic event in amyotrophic lateral sclerosis (ALS). Yet, direct links between NMJ pathways and…”
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4
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis
Published in Acta neuropathologica (01-06-2017)“…Motor neuron-extrinsic mechanisms have been shown to participate in the pathogenesis of ALS-SOD1, one familial form of amyotrophic lateral sclerosis (ALS). It…”
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5
Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis
Published in Brain (London, England : 1878) (01-04-2016)“…Amyotrophic lateral sclerosis, the most common adult-onset motor neuron disease, leads to death within 3 to 5 years after onset. Beyond progressive motor…”
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6
Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression
Published in Neurobiology of disease (01-05-2021)“…Large polyglutamine expansions in Ataxin-2 (ATXN2) cause multi-system nervous atrophy in Spinocerebellar Ataxia type 2 (SCA2). Intermediate size expansions…”
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7
Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
Published in Nature communications (21-05-2021)“…Gene mutations causing cytoplasmic mislocalization of the RNA-binding protein FUS lead to severe forms of amyotrophic lateral sclerosis (ALS). Cytoplasmic…”
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8
Degeneration of serotonin neurons triggers spasticity in amyotrophic lateral sclerosis
Published in Annals of neurology (01-09-2017)“…Objective Spasticity occurs in a wide range of neurological diseases, including neurodegenerative diseases, after trauma, and after stroke, and is…”
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9
Mutant FUS induces chromatin reorganization in the hippocampus and alters memory processes
Published in Progress in neurobiology (01-08-2023)“…Cytoplasmic mislocalization of the nuclear Fused in Sarcoma (FUS) protein is associated to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia…”
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10
Loss of hypothalamic MCH decreases food intake in amyotrophic lateral sclerosis
Published in Acta neuropathologica (01-06-2023)“…Amyotrophic lateral sclerosis (ALS) is associated with impaired energy metabolism, including weight loss and decreased appetite which are negatively correlated…”
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11
In‐depth analysis of data from the RAS‐ALS study reveals new insights in rasagiline treatment for amyotrophic lateral sclerosis
Published in European journal of neurology (01-04-2024)“…Background and purpose In 2016, we concluded a randomized controlled trial testing 1 mg rasagiline per day add‐on to standard therapy in 252 amyotrophic…”
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12
Serotonin 2B receptor slows disease progression and prevents degeneration of spinal cord mononuclear phagocytes in amyotrophic lateral sclerosis
Published in Acta neuropathologica (01-03-2016)“…Microglia are the resident mononuclear phagocytes of the central nervous system and have been implicated in the pathogenesis of neurodegenerative diseases such…”
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13
Low dietary protein content alleviates motor symptoms in mice with mutant dynactin/dynein-mediated neurodegeneration
Published in Human molecular genetics (15-04-2015)“…Mutations in components of the molecular motor dynein/dynactin lead to neurodegenerative diseases of the motor system or atypical parkinsonism. These mutations…”
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14
Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation
Published in Molecular neurodegeneration (06-09-2021)“…Mutations in FUS, an RNA-binding protein involved in multiple steps of RNA metabolism, are associated with the most severe forms of amyotrophic lateral…”
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15
Integrative genetic analysis illuminates ALS heritability and identifies risk genes
Published in Nature communications (20-01-2023)“…Amyotrophic lateral sclerosis (ALS) has substantial heritability, in part shared with fronto-temporal dementia (FTD). We show that ALS heritability is enriched…”
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16
Author Correction: Integrative genetic analysis illuminates ALS heritability and identifies risk genes
Published in Nature communications (04-12-2023)Get full text
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17
Mutant FUS induces chromatin reorganization in the hippocampus and alters memory processes
Published in Progress in neurobiology (01-08-2023)Get full text
Journal Article -
18
Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
Published in Nature communications (01-12-2021)“…Gene mutations causing cytoplasmic mislocalization of the RNA-binding protein FUS lead to severe forms of amyotrophic lateral sclerosis (ALS). Cytoplasmic…”
Get full text
Journal Article -
19
Evaluation of a 5-HT 2B receptor agonist in a murine model of amyotrophic lateral sclerosis
Published in Scientific reports (08-12-2021)“…Degeneration of brainstem serotonin neurons has been demonstrated in ALS patients and mouse models and was found responsible for the development of spasticity…”
Get full text
Journal Article