A5058509044- Prion Diseases and Protein Misfolding
- Neuroscience and Neural Engineering
- Neurological diseases and metabolism
- Neuroinflammation and Neurodegeneration Mechanisms
- 3D Printing in Biomedical Research
- Neuroscience and Neuropharmacology Research
- Neurogenesis and neuroplasticity mechanisms
- S100 Proteins and Annexins
- Alzheimer's disease research and treatments
- Conducting polymers and applications
- Immune cells in cancer
- Trace Elements in Health
- Genetic and Kidney Cyst Diseases
- RNA Research and Splicing
- Electrochemical Analysis and Applications
- Metabolism and Genetic Disorders
- RNA Interference and Gene Delivery
- Gestational Trophoblastic Disease Studies
- Anesthesia and Neurotoxicity Research
- Viral Infections and Immunology Research
- Force Microscopy Techniques and Applications
- Cell Image Analysis Techniques
- Hereditary Neurological Disorders
- Microtubule and mitosis dynamics
- Ziziphus Jujuba Studies and Applications
Inserm
2008-2024
Université de Lille
2020-2024
Centre Hospitalier Universitaire de Lille
2020-2024
Centre de Recherche Jean Pierre Aubert
2018-2023
Lille Neurosciences & Cognition
2020-2023
Virologie et Immunologie Moléculaires
2011-2022
Université Paris-Saclay
2016-2021
Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement
2021
Université de Versailles Saint-Quentin-en-Yvelines
2021
Institut National de la Recherche Agronomique
2014
l-2-Hydroxyglutaric aciduria (l-2-HGA) is characterized by progressive deterioration of central nervous system function including epilepsy and macrocephaly in 50% cases, elevated levels l-2-hydroxyglutaric acid urine, blood cerebrospinal fluid (CSF). Nuclear magnetic resonance imaging shows distinct abnormalities. We report the identification a gene for l-2-HGA (MIM 236792) using homozygosity mapping. Nine homozygous mutations three missense mutations, two nonsense splice site deletions were...
Abstract Caprine-like Generalized Hypoplasia Syndrome (SHGC) is an autosomal-recessive disorder in Montbéliarde cattle. Affected animals present a wide range of clinical features that include the following: delayed development with low birth weight, hind limb muscular hypoplasia, caprine-like thin head and partial coat depigmentation. Here we show SHGC caused by truncating mutation CEP250 gene encodes centrosomal protein C-Nap1. This results centrosome splitting, which neither affects...
ABSTRACT The dietary exposure of the human population to prions responsible for bovine spongiform encephalopathy (BSE) epizooty has led emergence variant Creutzfeldt-Jakob disease (vCJD). This fatal, untreatable neurodegenerative disorder is a growing public health concern because prevalence infection seems much greater than incidence and secondary transmission vCJD by blood transfusion or use products occurred. A current limitation in CJD risk assessment lack quantitative information on...
Abstract The prion protein is infamous for its involvement in a group of neurodegenerative diseases known as Transmissible Spongiform Encephalopathies. In the longstanding quest to decipher physiological function cellular isoform, PrPC, discovery participation self-renewal hematopoietic and neural stem cells has cast new spotlight on potential role cell biology. However, still little molecular mechanisms at play. Here, by combining vitro vivo murine models PrPC depletion, we establish that...
Microglial cells, as the primary defense line in central nervous system, play a crucial role responding to various mechanical signals that can trigger their activation. Despite extensive research on impact of chemical signaling brain understanding microglia remains limited. To bridge this gap, we subjected microglial cells singular stretch and compared responses with those induced by lipopolysaccharide treatment, well-established activator. Here show stretching leads activation, highlighting...
Guanabenz (GA) is an orally active α2-adrenergic agonist that has been used for many years the treatment of hypertension. We recently described GA also against both yeast and mammalian prions in receptor-independent manner. These data suggest this side-activity could be explored prion-based diseases other amyloid-based disorders. In perspective, potent antihypertensive activity happens to annoying side-effect limit its use. order get rid at receptors, we performed a structure-activity...
The biological function of the Prion protein remains largely unknown but recent data revealed its implication in early zebrafish and mammalian embryogenesis. To gain further insight into function, comparative transcriptomic analysis between FVB/N Prnp knockout mice was performed at embryonic stages. RNAseq differential expression 73 263 genes E6.5 E7.5, respectively. related metabolic pathways identified this partially overlap with those described PrP1 PrP2 knockdown embryos prion-infected...
The potential requirement of either the Prion or Shadoo protein for early mouse embryogenesis was recently suggested. However, current data did not allow to precise developmental process that affected in absence both proteins and led observed lethal phenotype. In present study, using various Prnp transgenic lines lentiviral vectors expressing shRNAs target Shadoo-encoding mRNA, we further demonstrate specific at least one these two PrP-related stages. Histological analysis reveals defect...
Alzheimer's disease (AD) is a neurodegenerative and the most frequent cause of dementia. It characterized by accumulation in brain two pathological protein aggregates: amyloid-β peptides (Aβ) abnormally phosphorylated tau. The progressive cognitive decline observed patients strongly correlates with synaptic loss. Many lines evidence suggest that soluble forms Aβ accumulate into where they synapse degeneration. Stopping their spreading and/or targeting pathophysiological mechanisms leading to...
Prion transmission can occur by blood transfusion in human variant Creutzfeldt-Jakob disease and experimental animal models, including sheep. Screening of its derivatives for the presence prions became therefore a major public health issue. As infectious titer is reportedly low, highly sensitive robust methods are required to detect derived products. The objectives this study were compare different - vitro, ex vivo assays prion infectivity cells prepared from samples obtained scrapie...
Abstract Although conversion of the cellular form prion protein (PrP C ) into a misfolded isoform is underlying cause diseases, understanding PrP physiological functions has remained challenging. depletion or overexpression alters proliferation and differentiation properties various types stem progenitor cells in vitro by unknown mechanisms. Such involvement remains uncertain vivo absence any drastic phenotype mice lacking . Here, we report enrichment at base primary cilium from central...
Deformation, compression, or stretching of brain tissues cause diffuse axonal injury (DAI) and induce structural functional alterations astrocytes, the most abundant cell type in brain. To gain further insight into role mechanically activated astrocytes on neuronal networks, this study was designed to investigate whether cytokines released by can affect growth synaptic connections cortical networks. Astrocytes were cultivated elastic membranes subjected repetitive mechanical insults, whereas...
Abstract Recently, the development of electronic devices to extracellularly record simultaneous electrical activities numerous neurons has been blooming, opening new possibilities interface and decode neuronal activity. In this work, we tested how use EDOT electropolymerization tune post-fabrication materials could optimize cell/electrode such devices. Our results showed an improved signal-to-noise ratio, better biocompatibility, a higher number detected in comparison with gold electrodes....
In naturally acquired transmissible spongiform encephalopathies, the pathogenic agents or prions spread from sites of initial peripheral uptake replication to brain where they cause progressive and fatal neurodegeneration. Routing via nervous system is considered be one main pathways central system. Replication in Schwann cells viewed as a potentially important mechanism for efficient prion along nerves. Here we used Cre-loxP mouse transgenetic approach disrupt host-encoded protein (PrP(C))...
Objective.Tau ablation has a protective effect in epilepsy due to inhibition of the hyperexcitability/hypersynchrony. Protection may also occur transgenic models Alzheimer's disease by reducing epileptic activity and normalizing excitation/inhibition imbalance. However, it is difficult determine exact functions tau, because tau knockout (tauKO) brain networks exhibit elusive phenotypes. In this study, we aimed further explore physiological role using network remodeling.Approach.The was...