Home > Granted research > 84 publications on granted projects
Date Revue Principal investigator Year
Sept-18 Human Molecular Genetics FUS-induced neurotoxicity in Drosophila is prevented by downregulating nucleocytoplasmic transport proteins L. Van den Bosch, Belgium 2013
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
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
Oct-16 SMALL GTPASES C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy K. De Vos, England 2012
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
Feb-14 Frontiers in Cellular NeuroScience A plural role for lipids in motoneurone disease J. P. Loeffler / P. F. Pradat, France 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
Sept-13 Frontiers in Cellular Neuroscience Fatting the brain: a brief of recent research J. P. Loeffler / P. F. Pradat, France 2009
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
May-13 Brain Is SOD1 loss of function involved in amyotrophic lateral sclerosis? E. Fisher, England 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