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Séance de cours

Maladies du motoneurone: physiopathologie et thérapies

Dans cours

BIO-480: Neuroscience: from molecular mechanisms to disease

The goal of the course is to guide students through the essential aspects of molecular neuroscience and neurodegenerative diseases. The student will gain the ability to dissect the molecular basis of disease in the nervous system in order to begin to under ...

Description

Cette séance de cours couvre le système moteur, les maladies du motoneurone et la sclérose latérale amyotrophique. Il explique le contrôle des mouvements volontaires, l'exécution des mouvements et le rôle des ganglions basaux dans le système moteur. La séance de cours se penche également sur l’organisation du système moteur, des motoneurones supérieurs et inférieurs et des voies descendantes de la moelle épinière. En outre, il discute du cortex moteur, de l'organisation de la moelle épinière chez les espèces de mammifères et du système neuromusculaire. La présentation se termine par un aperçu du circuit de la moelle épinière pour le contrôle du mouvement, des unités motrices et de la jonction neuromusculaire.

Enseignant·e·s (4)

Bernard Schneider

Johannes Gräff

Academic appointments 2013- Tenure Track Assistant Professor, Swiss Federal Institute of Technology (EPFL), Lausanne, Switzerland, School of Life Sciences, Brain Mind Institute. 2009-2013 Postdoctoral fellow, Massachusetts Institute of Technology (MIT), Cambridge, USA, Picower Institute for Learning and Memory. 2009 Postdoctoral associate, Swiss Federal Institute of Technology (ETHZ), Zürich, Switzerland, Brain Research Institute. Education 2009 PhD in Neurosciences, Swiss Federal Institute of Technology (ETHZ), Zürich, Switzerland, Department of Biology, Brain Research Institute. 2005 Diploma (M.Sc. equivalent) in Biological Sciences, University of Lausanne, Switzerland, Faculty of Biology and Medicine, Department of Ecology and Evolution. 1998 Matura, Gymnasium Type B, Kantonsschule am Burggraben St. Gallen, Switzerland.

Hilal Lashuel

2012-2013 Visiting Professor, Standford University. Stanford School of Medicine 2011- Associate Professor of Life Sciences-Brain Mind Institute-EPFL Dir. Laboratory of Chemical Biology of Neurodegeneration 2005-2011 Assistant Professor of Life Sciences-Brain Mind Institute-EPFL Dir. Laboratory of Molecular Neurobiology and Neuroproteomics 2005-2008 Director- EPFL Proteomic Core Facility 2002-2004 Instructor of Neurology- Harvard Medical School and Brigham and Women's Hospital 2001-2002 Sabbatical Fellow- Laboratory for Drug Discovery in Neurodegeneration Harvard Medical School, 2001-2002 Post-doctoral Fellow- Center for Neurologic Diseases Harvard Medical School and Brigham and Women's Hospital Advisor- Prof. Peter T. Lansbury 2000-2001 Research Scientist, The Picower Institute for Medical Research, Great Neck New York 1994-2000 PhD Student; Texas A&M University and the Scripps Research Institute Advisor- Prof. Jeffery W. Kelly 1990-1994 B.S. City University of New York, Brooklyn College Dr. Hilal A. Lashuel received his B.Sc. degree in chemistry from the City University of New York in 1994 and completed his doctoral studies at Texas A&M University and the Scripps Research Institute in 2000. After obtaining his doctoral degree, he became a research fellow at the Picower Institute for Medical Research in Long Island New York. In 2001, he moved to Harvard Medical School and the Brigham and Women's Hospital as a research fellow in the Center for Neurologic Diseases and was later promoted to an instructor in neurology at Harvard Medical School. During his tenure (2001-2004) at Harvard Medical School his work focused on understanding the mechanisms of protein misfolding and fibrillogenesis and the role of these processes in the pathogenesis of Parkinson's and Alzheimer's disease. In 2005 Dr. Lashuel moved Switzerland to join the Brain Mind Institute at the Swiss Federal Institute of Technology Lausanne as a tenure-track assistant professor in neurosciences. Currently, Dr. Lashuel is an associate professor of life sciences and the director of the laboratory of molecular and chemical biology of neurodegeneration. (http://lashuel-lab.epfl.ch/). Research efforts in the Lashuels laboratory focus on understanding the molecular mechanisms of neurodegeneration and developing novel strategies to diagnose and treat neurodegenerative diseases such as Alzheimers and Parkinsons disease. Research in the Lashuel lab is funded by several international funding agencies and foundations, including the Swiss National Science Foundation, European FP7 program (Marie Curie and ERC grants), Human Science Frontiers, Strauss Foundation, Cure the Huntingtons disease foundation and Michael J Fox foundation and is supported by collaborations with pharmaceutical and biotech companies (http://lashuel-lab.epfl.ch/page-50538-en.html), Nestle, Merck-Serono, AC Immune and Johnson and Johnson. Dr. Lashuels research has resulted in the characterization of novel quaternary structure intermediates on the amyloid pathway, identification of potential therapeutic targets, and new hypotheses concerning the mechanisms of pathogenesis in Alzheimers disease, Parkinsons disease and related disorders. Dr. Lashuel scientific contribution to this field includes i) more than100 publications in major peer reviewed journals including Nature journals, Cell, PNAS, JBC, J. Neuroscience JACS, and Angewandtie Chemie; ii) three patents on novel strategies for preventing protein aggregation and treating autoimmune and inflammatory diseases; iii) more than 150 invited lectures since 2002 and more than 5500 citations (7800 citation-Google Scholar) since 1996. Dr. Lashuel has received several pre-doctoral and post-doctoral awards and fellowships and was the recipient of two prestigious awards given to young investigators; Human Science Frontiers young investigator research award and the European Research Council (ERC) starting independent researcher grant and the ERC proof of concept award (2013) These awards provide more than $2.5 Million to Dr. Lashuel to translate some of his ideas and projects into novel strategies for diagnosing and treating neurodegenerative diseases such as Alzheimers and Parkinsons disease. Dr. Lashuel has chaired and co-organized several international conferences and serves as an academic editor for PLoS ONE, an associate editor for frontiers of molecular neuroscience, member of the Editorial advisory board of ChemBioChem and ad hoc reviewer for several international scientific journals and funding agencies.

Source officielle

Proximité ontologique

Biologie

Anatomie: Neuroanatomie

Concepts associés (32)

Motoneurone

Les motoneurones constituent la voie de sortie du système nerveux central ou la voie finale de tout acte moteur. Les corps cellulaires des motoneurones sont situés soit dans le tronc cérébral, soit dans la corne ventrale de la substance grise de la moelle épinière. Chaque motoneurone possède un axone qui part du système nerveux central pour innerver les fibres musculaires d'un muscle. L'ensemble constitué par un motoneurone et les fibres musculaires qu'il innerve constitue une unité motrice.

Alpha motor neuron

Alpha (α) motor neurons (also called alpha motoneurons), are large, multipolar lower motor neurons of the brainstem and spinal cord. They innervate extrafusal muscle fibers of skeletal muscle and are directly responsible for initiating their contraction. Alpha motor neurons are distinct from gamma motor neurons, which innervate intrafusal muscle fibers of muscle spindles. While their cell bodies are found in the central nervous system (CNS), α motor neurons are also considered part of the somatic nervous system—a branch of the peripheral nervous system (PNS)—because their axons extend into the periphery to innervate skeletal muscles.

Motor system

The motor system is the set of central and peripheral structures in the nervous system that support motor functions, i.e. movement. Peripheral structures may include skeletal muscles and neural connections with muscle tissues. Central structures include cerebral cortex, brainstem, spinal cord, pyramidal system including the upper motor neurons, extrapyramidal system, cerebellum, and the lower motor neurons in the brainstem and the spinal cord. The motor system is a biological system with close ties to the muscular system and the circulatory system.

Moelle spinale

La moelle spinale (selon la nouvelle nomenclature), ou moelle épinière (dans l’ancienne nomenclature), désigne la partie du système nerveux central qui prolonge la moelle allongée appartenant au tronc cérébral. Elle est contenue dans le canal rachidien (canal formé par la superposition des foramens vertébraux), qui la soutient et la protège. Elle est constituée de neurones et de cellules gliales. Sa fonction principale est la transmission des messages nerveux entre le cerveau et le reste du corps.

Cortex moteur

Le cortex moteur désigne l'ensemble des aires du cortex cérébral qui participent à la planification, au contrôle et à l'exécution des mouvements volontaires des muscles du corps. D'un point de vue anatomique, le cortex moteur est situé dans la partie postérieure du lobe frontal, au niveau de la région caudale de la circonvolution frontale ascendante en avant du sillon central. Le cortex moteur est en interaction constante avec d'autres structures nerveuses impliquées dans le mouvement comme le système des ganglions de la base et le cervelet.

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Séances de cours associées (39)

Contrôle neuronal du mouvement : systèmes sensorimoteurs

Explore le contrôle neuronal du mouvement, en mettant l'accent sur les systèmes sensorimoteurs, les circuits médullaires et les réflexes d'étirement musculaire.

Cerebelum: Contrôle neuronal des mouvements

Explore le contrôle neuronal du mouvement, en mettant l'accent sur le rôle du cervelet dans l'apprentissage et la coordination des moteurs.

Neuromodulation humaine : activité de la moelle épinière

Explore les méthodes avancées de neuromodulation humaine pour l'activité de la moelle épinière, y compris les applications cliniques et les mécanismes de lésions de la moelle épinière.

Anatomie cérébrale

Couvre les divisions du système nerveux, la structure du cerveau, les nerfs crâniens et les conditions cérébrales.

Maladies du motoneurone

Explore les maladies des motoneurones, en discutant de leur classification, des signatures pathologiques, de l'étiologie et des traitements potentiels, en mettant l'accent sur l'atrophie musculaire spinale.

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Concepts associés (32)