Accueil > Recherches financées > 106 Publications sur les projets financés
Date Journal Titre et Lien vers la publication Investigateur principal année de sélection du projet
avril-23 Acta Neuropathol Loss of hypothalamic MCH decreases food intake in amyotrophic lateral sclerosis L. Dupuis, France 2019
mars-23 EBioMedicine Metabolic alterations precede neurofilament changes in presymptomatic ALS gene carriers L. Dupuis, France 2019
oct-21 Int J Mol Sci Histone Deacetylase Inhibition Regulates Lipid Homeostasis in a Mouse Model of Amyotrophic Lateral Sclerosis L. van den Bosch, Belgique 2019
juin-21 EMBO Mol Med Synaptic disruption and CREB-regulated transcription are restored by K+ channel blockers in ALS L. Dupuis, France F. Roselli, Italie 2019
juin-21 Neurology Visualization of Gray Matter Atrophy and Anterior Corticospinal Tract Signal Hyperintensity in Amyotrophic Lateral Sclerosis Using 7T MRI V. Callot, France 2018
juin-21 Neurology Metabolic Brain Changes Across Different Levels Of Cognitive Impairment In ALS: A 18F-FDG-PET Study A. Chio, Italie 2016
mai-21 Transl Neurodegener Disruption of orbitofrontal-hypothalamic projections in a murine ALS model and in human patients L. Dupuis, France 2019
fev-21 Journal of Neural Engineering Sustained attention to response task-related beta oscillations relate to performance and provide a functional biomarker in ALS O. Hardiman, Irlande 2019
nov-20 J of Nucl Medecine Brain metabolic correlates of apathy in amyotrophic lateral sclerosis: An 18F-FDG-positron emission tomography study A. Chio, Italie 2016
oct-20 Eur J Nucl Med Mol Imaging Brain metabolic changes across King’s stages in amyotrophic lateral sclerosis: a 18F-2-fluoro-2-deoxy-d-glucose-positron emission tomography study A. Chio, Italie 2016
oct-20 Nature Neuroscience Modifying macrophages at the periphery has the capacity to change microglial reactivity and to extend ALS survival C. Lobsiger, France 2015
juin-20 Jal of Exp Medicine Synaptic restoration by cAMP/PKA drives activity-dependent neuroprotection to motoneurons in ALS D. Zytnicki, France 2018
mai-20 Jama Neurology Use of Multimodal Imaging and Clinical Biomarkers in Presymptomatic Carriers of C9orf72 Repeat Expansion A. Chio, Italie et P. Van Damme, Belgique 2016
avr-20 Cerebral Cortex Localization of Brain Networks Engaged by the Sustained Attention to Response Task Provides Quantitative Markers of Executive Impairment in Amyotrophic Lateral Sclerosis O. Hardiman, Irlande 2019
fev-20 Acta Neuropathologica Synaptotagmin 13 is neuroprotective across motor neuron diseases S. Corti, Italie et E. Hedlund, Suède 2018
fev-20 the EMBO journal Reduced autophagy upon C9ORF72 loss synergizes with dipeptide repeat protein toxicity in G4C2 repeat expansion disorders N. Charlet, France 2018
janv-20 Acta Neuropathologica Proteomics in cerebrospinal fluid and spinal cord suggests UCHL1, MAP2 and GPNMB as biomarkers and underpins importance of transcriptional pathways in amyotrophic lateral sclerosis M. Otto, Allemagne 2011
dec-19 Science Translational Medicine Human genetics and neuropathology suggest a link between miR-218 and amyotrophic lateral sclerosis pathophysiology E. Hornstein, Israël 2016
nov-19 The journal of Physiologie Absence of Hyperexcitability of Spinal Motoneurons in Patients With Amyotrophic Lateral Sclerosis D. Zytnicki, France 2016
juillet-19 Acta Neuropathologica Communication Restoration of histone acetylation ameliorates disease and metabolic abnormalities in a FUS mouse model L. Van den Bosch, Belgique 2016
mai-19 Advanced Biosystem In Vitro Modeling of Nerve–Muscle Connectivity in a Compartmentalized Tissue Culture Device B. Bryson, Angleterre 2014
janv-19 EBioMedicine Micro-RNAs secreted through astrocyte-derived extracellular vesicles cause neuronal network degeneration in C9orf72 ALS L. Ferraiuolo, Angleterre 2016
sept-18 Human Molecular Genetics FUS-induced neurotoxicity in Drosophila is prevented by downregulating nucleocytoplasmic transport proteins L. Van den Bosch, Belgique 2013
sept-18 SMALL GTPASES C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy K. De Vos, Angleterre 2012
août-18 Molecular Neurodegeneration Counteracting roles of MHCI and CD8+ T cells in the peripheral and central nervous system of ALS SOD1G93A mice G.Nardo, Italie 2013
juillet-18 Cell reports Molecular Dissection of FUS Points at Synergistic Effect of Low-Complexity Domains in Toxicity L. Van den Bosch, Belgique 2013
juillet-18 ALS Multicenter validation of [18F]-FDG PET and support-vector machine discriminant analysis in automatically classifying patients with amyotrophic lateral sclerosis versus controls A. Chio, Italie et P. Van Damme, Belgique 2016
juin-18 The EMBO journal Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis E. Buratti, Italie 2013
mai-18 Cell and Death disease Inhibiting p38 MAPK alpha rescues axonal retrograde transport defects in a mouse model of ALS G. Schiavo, Angleterre 2015
mai-18 The EMBO journal Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis E. Fisher, Angleterre 2009
mars-18 eLife Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS D. Zytnicki, France 2011
nov-17 EMBO Molecular Medicine G‐quadruplex‐binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo A. Isaacs, Angleterre 2013
nov-17 Genome research Detection of long repeat expansions from PCR-free whole-genome sequence data M. Van Es, Hollande 2012
oct-17 Nature Communications HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients L. Van den Bosch, Belgique 2016
sept-17 Genome research Detection of long repeat expansions from PCR-free whole-genome sequence data J. Veldink, Hollande 2010
juillet-17 Journal of Biological Chemistry Identification and characterization of Nanobodies targeting the EphA4 receptor P. Van Damme, Belgique 2013
juillet-17 Nucléic Acids Research Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells E. Buratti, Italie 2013
juillet-17 Journal of Biological Chemistry Point Mutations in TDP-43’ N-terminal Domain Compromise its Conformational Stability, Dimer Formation, Physiological Activities and Pathological Aggregation E. Buratti, Italie 2013
nov-16 Lancet Neurology Early diaphragm pacing in patients with amyotrophic lateral sclerosis (RespiStimALS): a randomised controlled triple-blind trial J. Gonzales, France 2011
oct-16 Journal of Neuroinflammation Immune response in peripheral axons delays disease progression in SOD1G93A mice G. Nardo, Italie 2013
sept-16 Acta Neuropathologica Peripheral monocytes are functionally altered and invade the CNS in ALS patients J. Weishaupt, Allemagne 2010
sept-16 Scientifc Reports TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins E. Buratti, Italie 2013
août-16 The EMBO journal The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy K. De Vos, Angleterre 2012
août-16 Acta Neuropathologica Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways J. Veldink, Hollande 2010
avril-16 Brain Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis L.Dupuis, France 2012
avril-16 FEBS The TDP-43 N-terminal domain structure at high resolution E. Buratti, Italie 2013
avril-16 PLOS Biology Electrostatic Repulsion Governs TDP-43 C-terminal Domain Aggregation E. Buratti, Italie 2013
mars-16 Acta Neuropathologica Serotonin 2B receptor slows disease progression and prevents degeneration of spinal cord mononuclear phagocytes in amyotrophic lateral sclerosis L.Dupuis, France 2012
mars-16 Acta Neuropathologica Presymptomatic activation of the PDGFCC pathway accelerates onset of ALS neurodegeneration U. Eriksson, Suède 2012
fév-16 Human Molecular Genetics TDP-43 functions within a network of hnRNP proteins to inhibit the production of a truncated human SORT1 receptor E. Buratti, Italie 2013
janv-16 Journal of Neurology Neurosurgery & Psychiatry Neurofilaments in the diagnosis of motoneuron diseases: a prospective study on 455 patients M. Otto, Allemagne 2011
janv-16 Annals of Neurology Neurofilament levels as biomarkers in asymptomatic and symptomatic familial amyotrophic lateral sclerosis M. Otto, Allemagne 2011
oct-15 Nucleic acids research TDP-43 affects splicing profiles and isoform production of genes involved in the apoptotic and mitotic cellular pathways E. Buratti, Italie 2013
sept-15 frontiers in Cellular Neuroscience Developing electrical properties of postnatal mouse D. Zytnicki, France 2011
août-15 Journal of Neurophysiology Potassium currents dynamically set the recruitment and firing properties of F- type motoneurons in neonatal mice D. Zytnicki, France 2011
juin-15 ALS and Frontotemporal Degeneration The longitudinal cerebrospinal fluid metabolomic profile of amyotrophic lateral sclerosis M. Turner, Angleterre 2012
juin-15 Neurobiology of disease Modeling amyotrophic lateral sclerosis in pure human iPSc-derived motor neurons isolated by a novel FACS double selection technique D. Bohl, France 2010
juin-15 The journal of Physical Chemistry Letters Structural Evidence of Amyloid Fibril Formation in the Putative Aggregation Domain of TDP-43 E. Buratti, Italie 2013
juin-15 Neurology Neurofilament light chain, A prognostic biomarker in amyotrophic lateral sclerosis M. Turner, Angleterre 2012
avr-15 Genomics Homozygosity mapping in an Irish ALS case-control cohort describes local demographic phenomena and points towards potential recessive risk loci 0. Hardiman, Irlande 2013
avr-15 Annals of Clinical and Translational Neurology CSF neurofilament light chain reflects corticospinal tract M. Turner, Angleterre 2012
avr-15 Neuroscience Motor neurons with differential vulnerability to degeneration show distinct protein signatures in health and ALS S. Corti, Italie 2012
fev-15 Neuropharmacology First-in-class thyrotropin-releasing hormone (TRH)-based compound binds to a pharmacologically distinct TRH receptor subtype in human brain and is effective in neurodegenerative models J. Kelly, Irlande 2013
fev-15 Neurobiolgy of Aging A second-generation Irish genome-wide association study for amyotrophic lateral sclerosis 0. Hardiman, Irlande 2013
janv-15 Prion The Structural Integrity of TDP-43 N-terminus is Required for Efficient Aggregate Entrapment and Consequent Loss of Protein Function E. Buratti, Italie 2013
janv-15 Brain System xC− is a mediator of microglial function and its deletion slows symptoms in amyotrophic lateral sclerosis mice S. Boillée, France 2009
janv-15 Annals of Clinical and Translational Neurology SOD1 silencing in motoneurons or glia rescues neuromuscular function in ALS mice C. Raoul, France /   P.Aebischer, Suisse 2009
janv-15 Human Molecular Genetics TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain E. Buratti, Italie 2013
janv-15 Cerebral Cortex Altered Functionality, Morphology, and Vesicular Glutamate Transporter Expression of Cortical Motor Neurons from a Presymptomatic Mouse Model of Amyotrophic Lateral Sclerosis D. Zytnicki, France 2011
oct-14 eLife Early intrinsic hyperexcitability does not contribute to motoneuron degeneration in amyotrophic lateral sclerosis D. Zytnicki, France 2011
sept-14 ALS Frontotemporal Degeneration Multicentre quality control evaluation of different biomarker candidates for amyotrophic lateral sclerosis M. Otto, Allemagne 2011
sept-14 ALS Frontotemporal Degeneration Limited Role of Free TDP-43 as a Diagnostic Tool in Neurodegenerative Diseases M. Otto, Allemagne 2011
août-14 Neurobiology of Aging Androgens affect muscle, motor neuron and survival in a mouse model of SOD1-related ALS M. Pennuto, Italie 2010
juil-14 Annals of Neurology C9orf72 and UNC13A…:  A genome-wide meta-analysis J. Veldink, Hollande 2010
juin-14 Neurobiology of Aging Widespread RNA metabolism impairment in sporadic inclusion body myositis TDP43-proteinopathy E. Fisher, Angleterre 2009
mai-14 EMBO reports Somatic and axonal LIGHT signaling elicit degenerative and regenerative responses in motoneurons, respectively C. Raoul, France /   P.Aebischer, Suisse 2009
avr-14 Journal of Physiology  Adult spinal motoneurones are not hyperexcitable in a mouse model of inherited amyotrophic lateral sclerosis , D. Zytnicki, France 2011
avr-14 Science  OPTICAL CONTROL OF MUSCLE FUNCTION BY TRANSPLANTATION OF STEM CELL–DERIVED MOTOR NEURONS IN MICE I. Lieberam, Angleterre 2010
mars-14 Archives of Biochemistry and Biophysics Structural characterization of the minimal segment of TDP-43 competent for aggregation E. Buratti, Italie 2013
févr-14 Human Gene Therapy Intracerebroventricular Injection of Adeno-Associated Virus 6 and 9 Vectors for Cell Type–Specific Transgene Expression in the Spinal Cord C. Raoul, France /      P.Aebischer, Suisse 2009
févr-14 Neurobiology of Disease Wild-type Cu/Zn superoxide dismutase stabilizes mutant variants by heterodimerization A. Clément / C. Behl, Allemagne 2009
janv-14 Neuroreport Cerebrospinal fluid-targeted delivery of neutralizing anti-IFNgamma antibody delays motor decline in an ALS mouse model C. Raoul, France / P. Aebischer, Suisse 20O9
janv-14 PLoS ONE A Nonsense Mutation in Mouse Tardbp Affects TDP43 Alternative Splicing Activity and Causes Limb-Clasping and Body Tone Defects E. Fisher, Angleterre 2009
nov.-13 Frontiers in Cellular Neuroscience Neuroimmunity dynamics and the development of therapeutic strategies for amyotrophic lateral sclerosis C. Raoul, France / P. Aebischer, Suisse 20O9
oct.-13 Human Molecular Genetics Loss and gain of Drosophila TDP-43 impair synaptic efficacy and motor control leading to age-related neurodegeneration by loss-of-function phenotypes. F. Hirth, Angleterre 2011
juil-13 Journal of Proteomics Angiogenin induces modifications in the astrocyte secretome: Relevance to amyotrophic lateral sclerosis J. H. Prehn, Irlande 2011
juin-13 PLoS ONE Systemic Down-Regulation of Delta-9 Desaturase Promotes Muscle Oxidative Metabolism and Accelerates Muscle Function Recovery following Nerve Injury J. P. Loeffler
/ P. F. Pradat, France
2009
mai-13 Human Molecular Genetics PGC-1α is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis P. Weydt, Allemagne 2009
févr-13 Brain Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity L. Dupuis, France 2012
févr-13 Human Molecular Genetics Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish T. Ramesh, Angleterre 2010
févr-13 Stem cells translational medicine Neural Progenitors Derived From Human Induced Pluripotent Stem Cells Survive and Differentiate Upon Transplantation Into a Rat Model of Amyotrophic Lateral Sclerosis D. Bohl, France 2010
janv-13 Human Molecular Genetics Loss and gain of Drosophila TDP-43 impair synaptic efficacy and motor control leading to age-related neurodegeneration by loss-of-function phenotypes F. Hirth, Angleterre 2011
janv-13 Cell reports TDP-43 Loss-of-Function Causes Neuronal Loss Due to Defective Steroid Receptor-Mediated Gene Program Switching in Drosophila F. Hirth, Angleterre 2011
août-12 Nature Medicine EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans P. Van
Damme, Belgique
2009
mai-12 European Journal of Neurology Elevated levels of IFNγ and LIGHT in the spinal cord of patients with sporadic amyotrophic lateral sclerosise C. Raoul, France / P. Aebischer, Suisse 2009
avr-12 PLoS ONE Mapping of Gene Expression Reveals CYP27A1 as a Susceptibility Gene for Sporadic ALS J. Veldink, Hollande 2010
avr-12 Neurobiology of Aging VCP mutations in familial and sporadic amyotrophic lateral sclerosis J. Veldink, Hollande 2010
avr-12 The Journal of Neuroscience Motoneurons Secrete Angiogenin to Induce RNA Cleavage in Astroglia J.H. Prehn, Irlande 2011
mars-12 Neurobiology of Aging UNC13A is a modifier of survival in amyotrophic lateral sclerosis J. Veldink, Hollande 2010
févr-12 Pharmacological Research The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis M. Pennuto, Italie 2010
janv-12 PLOS Genetics Unraveling the Regulatory Mechanisms Underlying Tissue-Dependent Genetic Variation of Gene Expression J.Veldink, Hollande 2010
nov-11 Human Molecular Genetics Mitochondrial redox signalling by p66Shc mediates ALS-like disease through Rac1 inactivation M.T. Carri, Italie 2009
mars-11 Disease Models &amp Mechanisms A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis E. Fisher, Angleterre 2009
nov-10 Cell Death and Differentiation IFNγ triggers a LIGHT-dependent selective death of motoneurons C. Raoul, France / P. Aebischer, Suisse 2009