Our laboratory aims at deciphering the pathological mechanisms that trigger Amyotrophic Lateral Sclerosis (ALS) and Fronto-Temporal Dementia, two closely related fatal and currently untractable neurodegenerative diseases. Our originality lies in the study of the reciprocal interactions that occur between the central nervous system and the peripheral organs, and the general aim of understanding the myriad of signs and symptoms associated with these diseases. For a detailed explanation of the research we develop, please visit the Research Themes. Luc Dupuis, Inserm U1118 Research Director
Thesis Defense - Gina Picchiarelli
Summer Break BBQ
3rd Price Bike Challenge
Promising first clinical trial
"Au Boulot à Vélo" Cycling Challenge
FRM team granted to Luc DUPUIS
Axa research fund on rare diseases granted to Luc DUPUIS
Second publication for 2018
Unit renew for 5 years !
First accepted paper for 2018 !
Our research unit is affiliated both to Inserm and the University of Strasbourg. It is located on the campus of the Faculty of Medicine, Strasbourg, and belongs to the Federation of Translational Medicine, and the NeuroPôle of Strasbourg.
Directed by Dr Luc Dupuis, Inserm Research Director, the lab permanent staffs is composed of an administrative assistant, six researchers (research director, research associate, professor and assistant professors), an engineer, three technicians, and two neurologists.
In addition, the lab also currently counts a post-doc, eight graduate students and two technicians. For details, please see Lab Members.
Our work would never be possible without the constant involvement of our permanent technical and administrative staff:
- Sylvie DIRRIG-GROSCH, Inserm Engineer, our specialist in microscopy,
- Annie PICCHINENNA, Technician, University of Strasbourg, who cares for our animals,
- Marie-José RUIVO, Technician, University of Strasbourg, who prepares our favourite buffers in her favourite dishes,
- Brigitte KUENEMANN, Administrative technician, University of Strasbourg, who links us to our institutional administrations.
Our team is involved in graduate students education. We welcome and train Master students in the fields of Neuroscience, Pharmacology and Physiopathology, as well as PhD students, in collaboration with the Doctoral School of Life Science and Health, Strasbourg.
We regularly benefit from the technical assistance of undergraduate students from the University of Strasbourg and the Lycée Jean-Rostand.
Our research relies on a multitude of complementary approaches, and benefits from dedicated Tools & Technologies.
Our work concentrates on neurodegenerative diseases, with a primary interest in two related diseases, Amyotrophic Lateral Sclerosis and Fronto-Temporal Dementia.
Amyotrophic Lateral Sclerosis (ALS) is the most frequent motor neuron degenerative disease, with 15 000 new patients each year in Europe and 140 000 worldwide. ALS develops as a progressive paralysis leading to death within 2 to 5 years of diagnosis. Frontotemporal Dementia (FTD) is the most frequent form of dementia after Alzheimer’s disease, and progresses to death within a few years following diagnosis. While these two diseases present with very different clinical signs, they may result from mutation affecting similar genes, and share similar risk factors. In spite of some 20 years of preclinical research, ALS and DFT remain totally incurable.
Our lab aims at developing new therapeutic strategies and identifying both diagnostic and prognostic biomarkers that may further help assessing the benefits of clinical trials conducted on ALS and DFT patients. Our work thus intersects the disciplines of fundamental research and translational research.
We develop 5 complementary research axes:
Characterization of the neural pathways involved in energy metabolism defects in ALS – Dr Luc DUPUIS
Cerebral cortex and corticospinal motor neurons in ALS – Dr Caroline ROUAUX
Development and characterization of new ALS and FTD animal models – Dr Frédérique RENE
Expertise
Mouse motor behavioural phenotyping;
Biochemistry and molecular biology: western blot, qPCR, ELISA;
Histology: immunostaining, histo-enzymology, in situ hybridization, retrograde labelling;
Transcriptomics;
Metabolomics;
Cell culture: primary cultures, cell lines, transfection, viral transduction, electroporation.
Equipment
Histology: vibratome, cryostat, microtome, ultramicrotome, hybridization oven;
Microscopy: fluorescence stereomicroscope, one regular upright fluorescence microscope and one upright fluorescence microscope equipped with a structured illumination system, one inverted fluorescence microscope;
Molecular biology and biochemistry: regular and quantitative thermal cyclers, Wester blot equipment;
Cell culture: dedicated and fully equipped TC room and L2 room;
Mouse phenotyping: dedicated phenotyping room, rotarod, catwalk, strength meter, openfield, electromyography;
Surgery: dedicated surgery room, stereotaxic frame, nanoinjector, electroporator.
Félicitations à Gina Picchiarelli qui vient de soutenir sa thèse après 4 ans de travail sur la relation entre muscle et SLA dans la mutation FUS.
"Rôle du muscle squelettique dans la sclérose latérale amyotrophique : apport de modèles transgéniques conditionnels"
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Congratulations to Gina Picchiarelli who has just successfully defend her thesis after 4 years working on the relationship between muscle and ALS involving the FUS mutation.
Traditionnellement avant les vacances d'été, l'équipe s'unit pour célébrer et se détendre autour d'un barbecue. Cette année, le temps était super et nous avons trouvé le meilleur endroit au soleil, le "Square Louise Weiss" à côté des fameux "Ponts couverts".
Bonne vacances d'été!
C’est dans la bonne humeur avec un fort esprit d’équipe que l’Unité 1118 s’est engagée dans le challenge "Au boulot à vélo" et pour sa première participation a décroché la 3 ème place ! Ces 2 semaines de challenge ont permis de mettre en évidence certains esprits de compétition mais surtout une cohésion et dynamique d’équipe. A l'année prochaine pour 1 mois complet de challenge !
Our Lab is participating to the Cycling challenge "Au Boulot à Vélo" (9th Edition)
We are proud to contribute and show how important it is to practice sport for good health.
After 2 weeks, we managed to ride together allmost 1600km and found all the 30 bike tags! See you next year.
Faculté de médecine - Bat 3 - 8ème Etage
11, rue Humann
67085 Strasbourg CEDEX
Last Main Publications
Vercruysse P, Vieau D, David Blum, Petersen A & Dupuis L
Hypothalamic alterations in neurodegenerative diseases and their relation to abnormal energy metabolism
Front. Mol. Neurosci. 2018 Jan | Accepted
Henriques A, Croixmarie V, Bouscary A, Mosbach A, Keime C, Boursier-Neyret C, Walter B, Spedding M & Loeffler J-P. Sphingolipid Metabolism Is Dysregulated at Transcriptomic and Metabolic Levels in the Spinal Cord of an Animal Model of Amyotrophic Lateral Sclerosis.
Front. Mol. Neurosci. 2018 Jan ; 10:433
Henriques A, Huebecker M, Blasco H, Keime C, Andres C, Corcia P, Priestman D, Platt F, Spedding M & Loeffler JP. Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis.
Scientific Reports. 2017 Jul ;7:5235.
Gorges M, Vercruysse P, Müller HP, Huppertz HJ, Rosenbohm A, Nagel G, Weydt P, Petersén Å, Ludolph AC, Kassubek J & Dupuis L.
Hypothalamic atrophy is related to body mass index and age at onset in amyotrophic lateral sclerosis
J Neurol Neurosurg Psychiatry. 2017 Jun ; Published Online First.
Scekic-Zahirovic J, El Oussini H, Mersmann S, Drenner K, Wagner M, Sun Y, Allmeroth K, Dieterlé S, Sinniger J, Dirrig-Grosch S, René F, Dormann D, Haass C, Ludolph AC, Lagier-Tourenne C, Storkebaum E & Dupuis L.
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.
Acta Neuropathologica. 2017 Feb ; 133(6):887-906.
Recently Defended PhD Theses
13/09/2013, Gina Picchiarelli
"Rôle du muscle squelettique dans la sclérose latérale amyotrophique : apport de modèles transgéniques conditionnels"
Dir : J.P. LOEFFLER & L. DUPUIS
25/09/2017, Christine MARQUES
"Dégénérescence des Neurones Moteurs Cortico-spinaux dans un modèle murin de Sclérose Latérale Amyotrophique : dynamique spatio-temporelle et mécanismes moléculaires"
Dir : J.P. LOEFFLER & C. ROUAUX
15/12/2016, Laura ROBELIN
"Analyse de la composition en acides gras des lipides dans la sclérose latérale amyotrophique : implications dans le processus pathologique"
Dir : J.P. LOEFFLER
12/07/2016, Hajer El OUSSINI-BEN CHAABANE
“Rôle des neurones sérotoninergiques sans la sclérose latérale amyotrophique”
Dir : L. DUPUIS
11/01/2016, Jelena SCEKIC-ZAHIROVIC
“Conditional truncation of the FUS protein in mice: a new animal model of the ALS/DFT continuum”
Dir : L. DUPUIS
04/12/2014, Aurélia VERNAY
“Caractérisation des souris CHMP2Bintron5, un modèle d'étude du continuum SLA-DFT”
Dir : F. RENE
04/12/2014, Lavinia PALAMIUC
“Etudes des altérations métaboliques musculaires au cours de la sclérose latérale amyotrophique : rôle dans le développement de la pathologie”
Dir : F. RENE
11/09/2013, Florent SCHMITT
“Rôle de la stéaroyl-coenzyme A désaturase 1, une enzyme de synthèse des acides gras mono-insaturés, dans un modèle transgénique d’étude de la Sclérose Latérale Amyotrophique”
Dir : J.P. LOEFFLER
09/06/2013, Ghulam HUSSAIN
“Role of stearoyl-coA desaturase-1 in maintaining muscle activity: study of a lesion model to understand the metabolic alternations characteristic of amyotrophic lateral sclerosis”
Dir : J.P. LOEFFLER
Local Institutional Partners
Collaborators
Local 
A.L. Boutillier, C. Mathis & J.C. Cassel, LNCA
C. Tranchant, HUS
E. Marchioni, IPHC
C. Gachet, EFS
J.P. Armspach & C. Po, iCube
I. Yalcin, INCI
IGBMC platforms
National 
C. Andres, Inserm U930, Tours
E. Kabashi, ICM/Inserm U975, Paris
L. Buée, Inserm U1172, Lille
P. Couratier, Inserm U1094, Limoges
L. Maroteaux, Inserm U839, Paris
G. Butler-Browne, Inst. Myologie, Paris
P.F. Pradat, APHP, Paris
P. Corcia, CHU Bretonneau, Tours
G. Butler-Browne, Inserm 7215, Paris
M. Spedding, SRS, Le Vesinet
International 
A.C. Ludolph, Ulm University, Germany
P. Van Damme, Leuven University, Belgium
A. Petersen, Lund University, Sweden
J.H. Veldink, UMC Utrecht, Netherlands
E. Storkebaum, Max Planck Institute, Germany
F. Platt, Oxford, UK
C. Lagier Tourenne, MGH, Boston, USA
D.W. Cleveland, UCSD, USA
H.D. Durham, McGill, Canada
M. Polyminidou, ETH Zurich, Switzerland
H. Durham, McGill University, Montreal, Canada
Maria Teresa Carri, Roma, Italy
Barry Boland, Cork, Ireland
Financial Supports
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