Eran Hornstein (Assoc. Prof., Dept. Molecular Genetics, Weizmann Institute, Israel) Proteomic mapping of SG disassembly processes suggests a potential neuroprotective role of SUMOylation in C9orf72-associated ALS
With a strong experimental basis at RNA molecular biology, Eran Hornstein and his lab are undertaking converging approaches towards uncovering the impact of RNA regulatory networks in neurodegeneration. Hornstein’s lab utilizes systems approaches, computational data science, mouse genetics and iPS cell-derived human neurons. Neuro-genetics: Non- protein coding regions, which encompass 97% of the human genome, are overlooked by traditional genetics studies. Hornstein’s lab is an affiliated member of the Project MinE consortium, exploring non-protein coding mechanisms. Hornstein’s lab also develops disease biomarkers based on cell-free RNA from patient biofluids. Neuro-cell biology: Membrane-less organelles, composed of RNA and protein partners, emerge as an important interface for RNA biology research in neurodegeneration. Hornstein’s lab is interested in understanding the potential connection between different types of membraneless organelles, normal neuron function and insufficiency in ALS. Eran Hornstein, MD, PhD is affiliated with the department of Molecular Genetics at the Weizmann Institute of Science, Israel, since 2006. He directs the Nella and Leon Benoziyo Center for Neurological Diseases and is incumbent of the Ira & Gail Mondry Professorial chair. For complete list of published work: Read More
Eran Perlson (Assoc. Prof., Dept Physiology & Pharmacol., Tel Aviv Univ., Israel) NMJ maintenance in ALS: the role of axoplasmic TDP-43 condensation in mitochondrial local protein synthesis
Prof. Eran Perlson is an Associate Professor in the Department of Physiology and Pharmacology at Tel-Aviv Faculty of Medicine and Sagol school of Neuroscience at Tel-Aviv University, Israel. He completed a B.Sc. in biological studies at Tel-Aviv University. His Ph.D. was obtained at the Weizmann Institute of Science in the laboratory of Prof. Mike Fainzilber on molecular mechanisms of axon regeneration after injury. He then joined the research group of Prof. Erika Holzbauer at University of Pennsylvania Medical School, in which he focused on understanding the mechanisms underlying axonal transport and neurodegenerative diseases. In October 2010, Eran returned to Tel-Aviv University and established his molecular neurodegeneration research group. His long-term research goal is to understand molecular mechanisms that play an essential role in motor neuron degeneration in ALS. Prof. Perlson and his team have developed a unique Lab-on-a-Chip platform that enables co-culture of motor neurons and muscle on a silicon chip. This platform mimics the motor unit and provides a simplified way to study mechanisms of neuromuscular junction formation, maintenance and disruption at subcellular levels. His scientific work has earned him a large number of distinguished grants, awards and honors, including the ERC starter grant, Human Frontier grant, Sieratzki-Korczyn Prize for Advances in Neuroscience; The Muscular Dystrophy Association, development grant; and The Milton Safenowitz Post-Doctoral Fellowship for ALS Research, ALSA. Eran is the author of numerous scientific publications and invited speaker to leading international meetings.
Eric Storkebaum (Head, Dept. Mol. Neurobiol. Radboud Univ. Netherlands) & Luc Dupuis (Director of Research INSERM UMR-S1118, Univ. Strasbourg, France): Investigating muscle-intrinsic toxicity of ALS-mutant FUS and its non-cell-autonomous contribution to motor neuron degeneration in a FUS-ALS knock-in mouse model
Dr. Erik Storkebaum is professor and chair of the Department of Molecular Neurobiology at the Donders Institute and Science Faculty of the Radboud University (Nijmegen, Netherlands). After his M.Sc. in pharmaceutical sciences (magna cum laude) at KU Leuven (Belgium), he obtained his PhD at VIB and KU Leuven, working on the role and therapeutic potential of vascular endothelial growth factor (VEGF) for ALS in Peter Carmeliet’s laboratory. During his postdoc in the laboratory of Patrick Callaerts (VIB and KU Leuven), he used the fruit fly Drosophila melanogaster as a genetic model for neuromuscular diseases. Before moving to Nijmegen in 2017, he was an independent ‘Max Planck Research Group Leader’ at the Max Planck Institute for Molecular Biomedicine in Muenster (Germany) from 2010 onward. Dr. Storkebaum received several awards, including the Pharmaleuven Prize (2001), the Galenus Prize (2005) and the Baron Simonart Prize (2005), and he was supported by multiple funding sources, including a Frick Foundation award in 2010 and an ERC Consolidator grant (2018). The Storkebaum lab combines Drosophila and mouse genetics to unravel molecular mechanisms underlying neuromuscular diseases, including ALS and Charcot-Marie-Tooth (CMT) peripheral neuropathy.
Dr. Luc Dupuis is Research Director at Inserm (French national institute for health and medical research) and is Head of the U1118 laboratory (Central and Peripheral Mechanisms of Neurodegeneration) in Strasbourg, France. He also coordinates the installation of the center for biomedical research in Strasbourg, a newly built research institute of 300 academic researchers.
Luc Dupuis co-authored more than 80 peer-reviewed publications in the best neuroscience and neurology journals, including Nature Neuroscience, Lancet Neurology, Brain, Annals of Neurology and Acta Neuropathologica, as well as in journals including PNAS and EMBO Journal. His work is entirely focused on ALS and is internationally recognized and he received several national and international awards (Fabrice le Mouhaer Award, Eliane et Gérard Pauthier Award, ENCALS Young Investigator Award 2013, Frick Foundation Award 2013), including the prestigious Bettencourt Schueller award “coup d’élan” in 2019. His lab provided evidence that weight loss is clinically and therapeutically relevant in ALS and has elucidated the underlying central mechanisms. They further characterized the role of serotonin neurons in ALS both in mouse models and patients. More recently, they characterized the mechanisms of FUS-mediated ALS by using conditional knock-in mice.
Dr. Patrick Dion is an Assistant Professor in the Department of Neurology and Neurosurgery at the Montreal Neurological Institute affiliated to McGill University. He obtained his M.Sc. and Ph.D. at Université de Sherbrooke (Sherbrooke, Canada) and Université de Montréal (Montreal, Canada), respectively, where he studied mechanisms of oncoviral cell transformation and the mutagenic specificity of alkylating agents at specific regions of the genome. His postdoctoral research was carried out at the Hospital for Sick Children of Toronto (Toronto, Canada) and later in the Centre for Neuroscience Research of McGill University (Montreal, Canada).
Prior to his move to the Neurological Institute in 2013, he was an Assistant Professor in the Department of Pathology and Cell Biology of Université de Montréal. Dr Dion is interested in the identification of novel genetic risk factors in a range of neurological conditions (amyotrophic lateral sclerosis, essential tremor, restless legs syndrome) as well as into the characterization of the underlying pathogenic events associated with these risk factors. He contributed to a number of studies in the field of ALS, and in 2015 his study of the nuclear pore component, Gle1, opened the door to a range of reports observing nuclear-cytoplasmic export defects where Gle1 and other component of the nuclear pore were observed to be mislocalized.
Dr. Guy Rouleau is the Director of the Montreal Neurological Institute and Hospital (The Neuro) affiliated to McGill University (Montreal, Canada). Over the last 25 years, Dr. Guy Rouleau and his team have focused on identifying the genes causing several neurological and psychiatric diseases, including autism, amyotrophic lateral sclerosis, hereditary neuropathies, epilepsy and schizophrenia, as well as providing a better understanding of the molecular mechanisms that lead to these disease symptoms. In 2017 he launched the Tanenbaum Open Science Institute (TOSI) aimed to accelerated the generation of knowledge and novel effective treatments for brain disorders by freeing science.
Dr. Rouleau’s main achievements are his contribution to the identification of dozens of disease causing genes and his discovery of new mutational mechanisms. In regard to ALS he has overseen the implementation of an iPSC facilities where assays and models are being studied to develop assays using cells from patients differentiated into disease relevant cell types. Dr. Rouleau has published nearly 800 articles in peer‐reviewed journals and has been quoted more than 65,000 times (Google Scholar). He has supervised more than a hundred students at the Masters, PhD and post‐doctoral levels in addition to receiving numerous awards for his contribution to science and society.
Dr. Daniel Zytnicki is a neurophysiologist. He performed his PhD work in the laboratory of Professor Y. Laporte at the Collège de France under the supervision of Léna Jami before moving for a post-doc to the University of Göteborg in the Lundberg's laboratory under the supervision of Elzbieta Jankowska. He was recruited in 1984 as a junior researcher at CNRS and since 1998, is a CNRS Research Director. He is currently working at the Paris Descartes University where he is leading the "Spinal Physiology and Pathophysiology team". In this University, he founded an interdisciplinary research unit, the Laboratory of Neurophysics and Physiology and he has directed the Research Federation in Neuroscience from 2014 to 2018.
His long-standing research work aims at elucidating 1°) how the proprioceptive inputs control the activity of motoneurons, i.e. the output stage of all motor systems, and 2°) the pathophysiological mechanisms that lead to degeneration of motoneurons in amyotrophic lateral sclerosis (ALS). He is an expert in electrophysiological studies in vivo. In particular, he applied the dynamic clamp technique to the in vivo investigation of spinal motoneurons, and he used this breakthrough to unravel how intrinsic conductances of motoneurons interplay to produce a physiologically consistent electrical activity. Together with Dr. Marin Manuel, he has developed an in vivo mouse preparation that allowed them to obtain very stable intracellular recordings in spinal motoneurons. Thanks to this preparation, they have investigated the intrinsic properties of spinal motoneurons from adult mice in normal state. Later on, they applied their expertise to investigate the properties of motoneurons and the spinal circuitry in ALS.Dr. Zytnicki has received in 2000 the Foulon prize from the French Academy of Sciences for his work on the proprioceptive control of motoneurons.
Dr. Francesco Roselli is Principal Investigator in the Department of Neurology at the University of Ulm. After receiving his medical degree and board qualification in neurology at Bari University School of Medicine, he was trained on the synaptic mechanisms of Alzheimer Disease in the laboratory of Prof. Osborne Almeida at the Max-Planck Institute of Psychiatry (obtaining his PhD in Experimental Neurobiology); he then joined the laboratory led of Prof. Pico Caroni at the Friedrich Miescher Institute in Basel, where he worked on chemogenetic manipulation of neurodegenerative pathways and on activity-dependent mechanisms of neuroprotection in amyotrophic lateral sclerosis.
As an independent investigator, he is pursuing the role of circuit and mesoscale factors in determining the vulnerability of neurons to different acute and chronic insults and he is exploring the manipulation of synaptic inputs to deliver neuroprotection. Dr. Roselli exploits a set of viral vectors, chemogenetic approaches, advanced imaging and multiple murine and iPSC models to investigate ALS, traumatic brain injury and collaborates extensively with partners in clinical neurology and in industry to bring the basic-science advancement to the bedside of patients.
He has received an EMBO long-term fellowship and research support from the Synapsis Foundation, the Thierry-Latran Foundation, the Baustein program of Ulm University-Medical faculty, the Deutsche Forschungsgemenischaft, the ERANET-NEURON program and from Hoffmann-La Roche and Bayer.
Prof. A.C. Kato
President of the Scientific Advisory Board
Faculty of Medicine , University of Geneva, 1211 Geneva 4, Switzerland
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