Home > Granted research > 106 publications on granted projects
Date Revue Principal investigator Year
april-23 Acta Neuropathol Loss of hypothalamic MCH decreases food intake in amyotrophic lateral sclerosis L. Dupuis, France 2019
march-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, Belgium 2019
june-21 EMBO Mol Med Synaptic disruption and CREB-regulated transcription are restored by K+ channel blockers in ALS L. Dupuis, France F. Roselli, Italy 2019
june-21 Neurology Visualization of Gray Matter Atrophy and Anterior Corticospinal Tract Signal Hyperintensity in Amyotrophic Lateral Sclerosis Using 7T MRI V. Callot, France 2018
june-21 Neurology Metabolic Brain Changes Across Different Levels Of Cognitive Impairment In ALS: A 18F-FDG-PET Study A. Chio, Italy 2016
may-21 Transl Neurodegener Disruption of orbitofrontal-hypothalamic projections in a murine ALS model and in human patients L. Dupuis, France 2019
feb-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, Ireland 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, Italy 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, Italy 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
june-20 Jal of Exp Medicine Synaptic restoration by cAMP/PKA drives activity-dependent neuroprotection to motoneurons in ALS D. Zytnicki, France 2018
May-20 Jama Neurology Use of Multimodal Imaging and Clinical Biomarkers in Presymptomatic Carriers of C9orf72 Repeat Expansion A. Chio, Italy and P. Van Damme, Belgium 2016
Apr-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, Ireland 2019
Feb-20 Acta Neuropathologica Synaptotagmin 13 is neuroprotective across motor neuron diseases S. Corti, Italy and E. Hedlund, Sweden 2018
Feb-20 the EMBO journal Reduced autophagy upon C9ORF72 loss synergizes with dipeptide repeat protein toxicity in G4C2 repeat expansion disorders N. Charlet, France 2018
Jan-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, Germanu 2011
Dec-19 Science Translational Medicine Human genetics and neuropathology suggest a link between miR-218 and amyotrophic lateral sclerosis pathophysiology E. Hornstein, Israel 2016
Nov-19 The journal of Physiologie Absence of Hyperexcitability of Spinal Motoneurons in Patients With Amyotrophic Lateral Sclerosis D. Zytnicki, France 2016
July-19 Acta Neuropathologica Communication Restoration of histone acetylation ameliorates disease and metabolic abnormalities in a FUS mouse model L. Van den Bosch, Belgium 2016
May-19 Advanced Biosystem In Vitro Modeling of Nerve–Muscle Connectivity in a Compartmentalized Tissue Culture Device B. Bryson, Great Britain 2014
Jan-19 EBioMedicine Micro-RNAs secreted through astrocyte-derived extracellular vesicles cause neuronal network degeneration in C9orf72 ALS L. Ferraiuolo,Great Britain 2016
Sept-18 Human Molecular Genetics FUS-induced neurotoxicity in Drosophila is prevented by downregulating nucleocytoplasmic transport proteins L. Van den Bosch, Belgium 2013
Sept-18 SMALL GTPASES C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy K. De Vos, Great Britain 2012
August-18 Molecular Neurodegeneration Counteracting roles of MHCI and CD8+ T cells in the peripheral and central nervous system of ALS SOD1G93A mice G.Nardo, Italy 2013
July-18 Cell reports Molecular Dissection of FUS Points at Synergistic Effect of Low-Complexity Domains in Toxicity L. Van den Bosch, Belgium 2013
July-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, Italy and P. Van Damme, Belgium 2016
June-18 The EMBO journal Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis E. Buratti, Italy 2013
May-18 Cell and Death disease Inhibiting p38 MAPK alpha rescues axonal retrograde transport defects in a mouse model of ALS G. Schiavo, Great Britain 2015
May-18 The EMBO journal Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis E. Fisher, England 2009
March-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, Great Britain 2013
Nov-17 Genome research Detection of long repeat expansions from PCR-free whole-genome sequence data M. Van Es, The Netherlands 2012
Oct-17 Nature Communications HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients L. Van den Bosch, Belgium 2016
Sept-17 Genome research Detection of long repeat expansions from PCR-free whole-genome sequence data J. Veldink, The Netherlands 2010
July-17 Journal of Biological Chemistry Identification and characterization of Nanobodies targeting the EphA4 receptor P. Van Damme, Belgium 2013
July-17 Nucléic Acids Research Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells E. Buratti, Italy 2013
July-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, Italy 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, Italy 2013
Sept-16 Acta Neuropathologica Peripheral monocytes are functionally altered and invade the CNS in ALS patients J. Weishaupt, Germany 2010
Sept-16 Scientifc Reports TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins E. Buratti, Italy 2013
Aug-16 The EMBO journal The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy K. De Vos, England 2012
Aug-16 Acta Neuropathologica Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways J. Veldink, The Netherlands 2010
Apr-16 Brain Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis L.Dupuis, France 2012
Apr-16 FEBS The TDP-43 N-terminal domain structure at high resolution E. Buratti, Italy 2013
Apr-16 PLOS Biology Electrostatic Repulsion Governs TDP-43 C-terminal Domain Aggregation E. Buratti, Italy 2013
March-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
March-16 Acta Neuropathologica Presymptomatic activation of the PDGFCC pathway accelerates onset of ALS neurodegeneration U. Eriksson, Sweden 2012
Feb-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, Italy 2013
Jan-16 Journal of Neurology Neurosurgery & Psychiatry Neurofilaments in the diagnosis of motoneuron diseases: a prospective study on 455 patients M. Otto, Germany 2011
Jan-16 Annals of Neurology Neurofilament levels as biomarkers in asymptomatic and symptomatic familial amyotrophic lateral sclerosis M. Otto, Germany 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, Italy 2013
Sept-15 frontiers in Cellular Neuroscience Developing electrical properties of postnatal mouse D. Zytnicki, France 2011
Aug-15 Journal of Neurophysiology Potassium currents dynamically set the recruitment and firing properties of F- type motoneurons in neonatal mice D. Zytnicki, France 2011
June-15 ALS and Frontotemporal Degeneration The longitudinal cerebrospinal fluid metabolomic profile of amyotrophic lateral sclerosis M. Turner, England 2012
June-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
June-15 The journal of Physical Chemistry Letters Structural Evidence of Amyloid Fibril Formation in the Putative Aggregation Domain of TDP-43 E. Buratti, Italy 2013
June-15 Neurology Neurofilament light chain, A prognostic biomarker in amyotrophic lateral sclerosis M. Turner, England 2012
Apr-15 Genomics Homozygosity mapping in an Irish ALS case-control cohort describes local demographic phenomena and points towards potential recessive risk loci 0. Hardiman, Ireland 2013
Apr-15 Annals of Clinical and Translational Neurology CSF neurofilament light chain reflects corticospinal tract M. Turner, England 2012
Apr-15 Neuroscience Motor neurons with differential vulnerability to degeneration show distinct protein signatures in health and ALS S. Corti, Italy 2012
Feb-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, Ireland 2013
Feb-15 Neurobiolgy of Aging A second-generation Irish genome-wide association study for amyotrophic lateral sclerosis 0. Hardiman, Ireland 2013
Jan-15 Prion The Structural Integrity of TDP-43 N-terminus is Required for Efficient Aggregate Entrapment and Consequent Loss of Protein Function E. Buratti, Italy 2013
Jan-15 Brain System xC− is a mediator of microglial function and its deletion slows symptoms in amyotrophic lateral sclerosis mice S. Boillée, France 2009
Jan-15 Annals of Clinical and Translational Neurology SOD1 silencing in motoneurons or glia rescues neuromuscular function in ALS mice C. Raoul, France /   P.Aebischer, Switzerland 2009
Jan-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, Italy 2013
Jan-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, Germany 2011
Sept-14 ALS Frontotemporal Degeneration Limited Role of Free TDP-43 as a Diagnostic Tool in Neurodegenerative Diseases M. Otto, Germany 2011
Aug-14 Neurobiology of Aging Androgens affect muscle, motor neuron and survival in a mouse model of SOD1-related ALS M. Pennuto, Italy 2010
Jul-14 Annals of Neurology C9orf72 and UNC13A…:  A genome-wide meta-analysis J. Veldink, The Netherlands 2010
June-14 Neurobiology of Aging Widespread RNA metabolism impairment in sporadic inclusion body myositis TDP43-proteinopathy E. Fisher, England 2009
May-14 EMBO reports Somatic and axonal LIGHT signaling elicit degenerative and regenerative responses in motoneurons, respectively C. Raoul, France /   P.Aebischer, Switzerland 2009
Apr-14 Journal of Physiology  Adult spinal motoneurones are not hyperexcitable in a mouse model of inherited amyotrophic lateral sclerosis , D. Zytnicki, France 2011
Apr-14 Science  OPTICAL CONTROL OF MUSCLE FUNCTION BY TRANSPLANTATION OF STEM CELL–DERIVED MOTOR NEURONS IN MICE I. Lieberam, England 2010
March-14 Archives of Biochemistry and Biophysics Structural characterization of the minimal segment of TDP-43 competent for aggregation E. Buratti, Italy 2013
Feb-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, Switzerland 2009
Feb-14 Neurobiology of Disease Wild-type Cu/Zn superoxide dismutase stabilizes mutant variants by heterodimerization A. Clément / C. Behl, Germany 2009
Jan-14 Neuroreport Cerebrospinal fluid-targeted delivery of neutralizing anti-IFNgamma antibody delays motor decline in an ALS mouse model C. Raoul, France / P. Aebischer, Switzerland 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, England 2009
Nov-13 Frontiers in Cellular Neuroscience Neuroimmunity dynamics and the development of therapeutic strategies for amyotrophic lateral sclerosis C. Raoul, France / P. Aebischer, Switzerland 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, England 2011
Jul-13 Journal of Proteomics Angiogenin induces modifications in the astrocyte secretome: Relevance to amyotrophic lateral sclerosis J. H. Prehn, Ireland 2011
June-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
May-13 Human Molecular Genetics PGC-1α is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis P. Weydt, Germany 2009
Feb-13 Brain Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity L. Dupuis, France 2012
Feb-13 Human Molecular Genetics Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish T. Ramesh, England 2010
Feb-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
Jan-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, England 2011
Jan-13 Cell reports TDP-43 Loss-of-Function Causes Neuronal Loss Due to Defective Steroid Receptor-Mediated Gene Program Switching in Drosophila F. Hirth, England 2011
Aug-12 Nature Medicine EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans P. Van
Damme, Belgium/td>		 2009
May-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
Apr-12 PLoS ONE Mapping of Gene Expression Reveals CYP27A1 as a Susceptibility Gene for Sporadic ALS J. Veldink, The Netherlands 2010
Apr-12 Neurobiology of Aging VCP mutations in familial and sporadic amyotrophic lateral sclerosis J. Veldink, The Netherlands 2010
Avr-12 The Journal of Neuroscience Motoneurons Secrete Angiogenin to Induce RNA Cleavage in Astroglia J.H. Prehn, Ireland 2011
March-12 Neurobiology of Aging UNC13A is a modifier of survival in amyotrophic lateral sclerosis J. Veldink, The Netherlands 2010
Febr-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, Italy 2010
Jan-12 PLOS Genetics Unraveling the Regulatory Mechanisms Underlying Tissue-Dependent Genetic Variation of Gene Expression J.Veldink, The Netherlands 2010
Nov-11 Human Molecular Genetics Mitochondrial redox signalling by p66Shc mediates ALS-like disease through Rac1 inactivation M.T. Carri, Italy 2009
March-11 Disease Models &amp Mechanisms A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis E. Fisher, England 2009
Nov-10 Cell Death and Differentiation IFNγ triggers a LIGHT-dependent selective death of motoneurons C. Raoul, France / P. Aebischer, Switzerland 2009
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