• A word from the Director

    The work we carry aims at deciphering the pathological mechanisms that trigger Amyotrophic Lateral Sclerosis (ALS) and related neurodegenerative diseases such as Fronto-Temporal Dementia. Our originality lies in the study of the reciprocal interactions that occur between the central nervous system and the peripheral organs, with an emphasis on the metabolic component these diseases. For a detailed explanation of the research we develop, please visit the Research Themes. Jean-Philippe Loeffler, Inserm U1118 Research Director

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 Jean-Philippe Loeffler, 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

Identification of biomarkers for ALS and FTD via high-throughput screening – Dr Jose-Luis GONZALEZ DE AGUILAR

Cerebral cortex and corticospinal motor neurons in ALS – Dr Caroline ROUAUX

Characterization of the peripheral alterations in ALS and FTD, with a focus on the neuromuscular junctions – Dr Jean-Philippe LOEFFLER

Development and characterization of new ALS and FTD animal models – Dr Frédérique RENE

PhD student

Phone:
Adress:

PhD student

Phone:
Adress:

Associate Professor, University of Strasbourg

Phone:
Adress:

Research Assistant

Phone:
Adress:

Inserm Engineer

Phone:
Adress:

Research Director, Inserm

Phone:
Adress:

Research Assistant

Phone:
Adress:

Assistant professor, University of Strasbourg

Phone:
Adress:

Research Assistant

Phone:
Adress:

Visiting scientist, Spedding Research Solutions

Phone:
Adress:

Administrative technician, University of Strasbourg

Phone:
Adress:

Professor, University of Strasbourg

Phone:
Adress:

Inserm Research Director

Phone:
Adress:

Postdoctoral Research Fellow

Phone:
Adress:

Assistant professor, University of Strasbourg

Phone:
Adress:

PhD student

Phone:
Adress:

PhD student

Phone:
Adress:

Technician

Phone:
Adress:

Assistant professor, University of Strasbourg

Phone:
Adress:

Research Associate, Inserm

Phone:
Adress:

Technician

Phone:
Adress:

PhD student

Phone:
Adress:

Postdoctoral Research Fellow

Phone:
Adress:

MD

Phone:
Adress:

Research Assistant

Phone:
Adress:

PhD student

Phone:
Adress:

Phone:
Email:
Adress:

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.

17/10/2017

Christine MARQUES & Gina PICCHIARELLI from our lab were awarded for the relevance of their oral and written presentation.

See the whole article here: PRIX JEUNES CHERCHEURS ARSLA 2017

12/07/2017

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.

21/06/2016

Vernay A, Therreau L, Blot B, Risson V, Dirrig-Grosch S, Waegaert R, Lequeu T, Sellal F, Schaeffer L, Sadoul R, Loeffler JP & René F.

A transgenic mouse expressing CHMP2Bintron5 mutant in neurons develops histological and behavioural features of amyotrophic lateral sclerosis and frontotemporal dementia.

Hum Mol Genet. 2016 Jun ; in press

16/03/2016

A new study from our lab showing the existence of hypothalamic alterations in amyotrophic lateral sclerosis patients and mouse models. This has important implications for treating pharmacologically weight loss.

Congratulations to Pauline as first author of the paper!

http://brain.oxfordjournals.org/cgi/content/full/aww004?ijkey=2XCZwVuqdEFfuFx&keytype=ref

01/01/2016

2016, already 3 articles in press

Vercruysse P, Sinniger J, El Oussini H, Scekic-Zahirovic J, Dieterlé S, Dengler R, Meyer T, Zierz S, Kassubek J, Fischer W, Dreyhaupt J, Grehl T, Hermann A, Grosskreutz J, Witting A, Van Den Bosch L, Spreux-Varoquaux O, Ludolph A.C. & Dupuis L. Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis, Brain. 2016, in press

Unité 1118

Faculté de médecine - Bat 3 - 8ème Etage
11, rue Humann
67085 Strasbourg CEDEX

 

 

Find the best public transport to come to our place thanks to the Vialsace website.

 

You will find more infos about Tram or Bus in the Strasbourg public transportation carrier website.

 

Last Main Publications

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

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

                         

                    

                    

                              

The website www.u1118.inserm.fr is edited by l'unité de recherche Inserm 1118,

Faculté de médecine
Bâtiment 3
11 rue Humann
67085 Strasbourg Cedex
Phone : 03 68 85 31 72

Publishing director : Jean-Philippe Loeffler, directeur de l'unité 1118.
Webmaster : Mathieu Fischer (contacter le webmestre)
Hosting : agence Tiz

Use of this site is reserved for personal purposes. Access to the site may be terminated at any time without notice, especially for reasons of force majeure or maintenance, or if the publisher decides to suspend or terminate the provision of this service. 

The publisher undertakes to ensure to the best of its abilities the accuracy and up-to-date nature of the information provided at the time of publication on the website. However, it does not guarantee the accuracy, accuracy or completeness of the information provided. The information on this site is non-contractual and may be changed at any time. 

This site is governed by French law. Foreign users formally accept the application of French law by visiting this site and using all or part of the site's features. 

Intellectual property 
The contents which appear or are available on the site are protected by copyright and intellectual property and are the exclusive property of the publisher. As such, you undertake not to copy, translate, reproduce, sell, publish, exploit or disseminate the site contents protected by intellectual property rights without prior written permission from the publisher. 

Private data 
The publisher undertakes not to disclose information communicated by internet users to third parties. These are confidential and will only be used for the purposes of the service. The site complies the regulations relating to data, files and freedoms as defined by Law No. 78-17, as amended. In respect of personal data collected during the site visit, the internet user has a right to access, modify, rectify and delete data in accordance with French law. These rights can be exercised by contacting the publisher at Unit 1122 at the address given above 

Links 
The site may contain links to websites operated by partners or third parties. Inserm has no way to control these sites and offers no warranty for these sites in terms of compliance with the laws and regulations in force. 

Canvassing alert 
The publisher does not sell advertising space in the publication. Any initiative for this purpose constitutes fraud and in contravention of the publisher’s rights.

Website by :

104, rue de Hochfelden
67200 STRASBOURG
Phone : 03 67 10 18 50
www.tiz.fr

In collaboration with the Délégation régionale Grand-Est of the Inserm and the unit 1118 of the Inserm.

Photo credits:
Inserm/P. Delapierre, ...

Contact : Inserm image bank  : www.serimedis.inserm.fr