A5033103154- Amyotrophic Lateral Sclerosis Research
- Neurological diseases and metabolism
- Neurogenetic and Muscular Disorders Research
- Mitochondrial Function and Pathology
- Prion Diseases and Protein Misfolding
- Parkinson's Disease Mechanisms and Treatments
- Hereditary Neurological Disorders
- Alcoholism and Thiamine Deficiency
- Neurological and metabolic disorders
- Genetic Neurodegenerative Diseases
- Advanced Fluorescence Microscopy Techniques
- Cell Image Analysis Techniques
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Infectious Encephalopathies and Encephalitis
- Photoacoustic and Ultrasonic Imaging
- Advanced Neuroimaging Techniques and Applications
- Health, Medicine and Society
- Alzheimer's disease research and treatments
- Cardiomyopathy and Myosin Studies
- Heavy Metal Exposure and Toxicity
- Dermatological and Skeletal Disorders
- Clinical Reasoning and Diagnostic Skills
- ATP Synthase and ATPases Research
- Peripheral Neuropathies and Disorders
- Glioma Diagnosis and Treatment
Inserm
2016-2024
Centre National de la Recherche Scientifique
2016-2024
Centre Hospitalier Universitaire d'Angers
2013-2024
Université d'Angers
2016-2024
Mitochondrial and Cardiovascular Physiopathology
2019-2021
Trialog (France)
2020
Centre Hospitalier Universitaire Amiens-Picardie
2020
Pitié-Salpêtrière Hospital
2015
Sorbonne Université
2015
Abstract Background With the lack of effective therapy, chemoprevention, and vaccination against SARS-CoV-2, focusing on immediate repurposing existing drugs gives hope curbing COVID-19 pandemic. A recent unbiased genomics-guided tracing SARS-CoV-2 targets in human cells identified vitamin D among three top-scoring molecules manifesting potential infection mitigation patterns. Growing pre-clinical epidemiological observational data support this assumption. We hypothesized that...
The cytoplasmic aggregation of TAR DNA-binding protein-43 (TDP-43) is a hallmark degenerating neurons in amyotrophic lateral sclerosis (ALS) and subsets frontotemporal dementia (FTD). In order to reduce TDP-43 pathology, we generated single-chain (scFv) antibodies against the RNA recognition motif 1 (RRM1) TDP-43, which involved abnormal protein self-aggregation interaction with p65 NF-κB. Virus-mediated delivery into nervous system scFv antibody, named VH7Vk9, reduced microgliosis mouse...
To determine whether hydroxychloroquine decreases the risk of adverse outcome in patients with mild to moderate coronavirus disease 2019 (COVID-19) at high worsening.We conducted a multicentre randomized double-blind placebo-controlled trial evaluating COVID-19 least one following factors for worsening: need supplemental oxygen, age ≥75 years, between 60 and 74 years presence co-morbidity. Severely ill requiring oxygen therapy >3 L/min or intensive care were excluded. Eligible 1:1 ratio...
Histological analysis of brain tissue samples provides valuable information about the pathological processes leading to common neurodegenerative disorders. In this context, development novel high-resolution imaging approaches is a current challenge in neuroscience.
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), 2 incurable neurodegenerative disorders, share the same pathological hallmark named TDP43 (TAR DNA binding protein 43) proteinopathy. This event is characterized by a consistent cytoplasmic mislocalization aggregation of TDP43, which loses its physiological properties, leading neurons to death. Antibody-based approaches are now emerging interventions in field disorders. Here, we tested target specificity, vivo...
Abstract Background The architecture and composition of glial (GCI) neuronal (NCI) α‐synuclein inclusions observed in multiple system atrophy (MSA) remain to be precisely defined better understand the disease. Methods Here, we used stochastic optical reconstruction microscopy (STORM) characterize nanoscale organization cryopreserved brain sections from MSA patients. Results STORM revealed a dense cross‐linked internal structure all GCI NCI. hyperphosphorylated (p‐αSyn) was similar cells,...
Objective: Sporadic amyotrophic lateral sclerosis (sALS) is a fatal neurodegenerative disorder affecting upper and lower motor neurons. In view of the heterogeneous presentation disease, one current challenges to identify diagnostic prognostic markers in order diagnose sALS at early stage stratify patients trials. this study, we sought cytological hallmarks patient-derived fibroblasts with aim finding new clinical-related disease. Methods: Primary were prospectively collected from affected...
Abstract The centrosome, as the main microtubule organizing centre, plays key roles in cell polarity, genome stability and ciliogenesis. recent identification of ribosomes, RNA-binding proteins transcripts at centrosome suggests local protein synthesis. In this context, we hypothesized that TDP-43, a highly conserved RNA binding involved pathophysiology amyotrophic lateral sclerosis frontotemporal lobar degeneration, could be enriched organelle. Using dedicated high magnification...
Abstract Introduction Therapeutic plasma exchange (TPE) can be proposed in the treatment of chronic dysimmune peripheral neuropathies (CDPN). Actual guidelines are however based on few studies, and indications protocols still remain to clarified. We conducted a 10‐year retrospective study order assess effectiveness tolerance TPE CDPN. Methods All patients treated for CDPN with from October 2006 March 2016 university hospital Angers were included. Patients considered responders when they...
Charcot-Marie-Tooth type 2A disease (CMT2A) is an inherited peripheral neuropathy mainly caused by mutations in the MFN2 gene coding for mitochondrial fusion protein mitofusin 2. Although a dominant fashion, few cases of early-onset autosomal recessive CMT2A (AR-CMT2A) have been reported recent years. In this study, we characterized structure network cultured primary fibroblasts obtained from AR-CMT2A family members. The patient-derived cells showed increase with large connected networks and...
The identification of biomarkers for amyotrophic lateral sclerosis (ALS) is a central issue in disease research. In recent article, Chatterjee et al. show that blood extracellular vesicles (EVs) with high levels transactive response DNA-binding protein 43 (TDP-43) accurately discriminate patients ALS from controls and correlate severity, providing promising biomarker early diagnosis monitoring.
Abstract In this study, we evaluated workshops with simulated patients and peers to improve English-speaking confidence among medical students. Results showed reduced apprehension when interviewing patients, high course satisfaction, perceived progress in English proficiency, increased future interactions supporting simulation-based methods education.
Hereditary optic neuropathies, including dominant atrophy and Leber's hereditary neuropathy, are genetic disorders characterized by retinal ganglion cell degeneration leading to vision loss, mainly associated with mitochondrial dysfunction. In this study, we analysed distribution ultrastructure in the retina longitudinal nerve sections of pre-symptomatic neuropathies mouse models Opa1 Nd6 deficiency identify early changes. Our results show significant fragmentation increased mitophagy