T3S-1124 - UMR-S 1124
Université de Paris
Campus Saint-Germain-des-Près
45 rue des Saints Pères
75270 Paris Cedex 06
Fax : +33 (0) 1 42 86 38 68
Pr. Frédéric Charbonnier
University Professor
Degeneration and plasticity of the locomotor system
frederic.charbonnier@-Code to remove to avoid SPAM-u-paris.fr
+33 1 42 86 40 37, room H330
Student :
Frederic Charbonnier obtained a PhD degree in Cellular and Molecular Biology at University Paris Descartes in 1998 and State Doctorate from University of Evry (France) in 2001. From 1998 to 1999 he was a post-doctoral fellow at the Neurosciences Institute of Fer-à-Moulin (France). From 1999 to 2005 he was an Associate Professor in Sport and Exercise Science at University of Evry (France). From 2005, he became a Full Professor at University Paris Descartes and currently heads the team “Neuromuscular degeneration and plasticity” at INSERM UMR_S1124. From January 2014, he was also nominated as head the Interdisciplinary Center of Chemistry Biology- Paris (CICB-Paris).
The group of Prof. Charbonnier develops original multidisciplinary approaches to describe and quantify the motor capacities in mice using behavioral, cellular and molecular analyses. The studies are focused mainly on the effects of the modulation of motor activities in mouse models of severe human neuromuscular diseases such as ALS and SMA.The team’s recent data obtained with the severe SMA mouse model clearly indicate that 1) motor unit maturation is delayed in SMA, 2) the importance of this delay is associated with the severity of neuronal death, and 3) the increase in motor neuron activity through appropriately designed exercise training programs limits developmental delay and neuronal death. These improvements lead to an increase in the motor capacities and, ultimately, to increased survival in SMA mice. The post-natal maturation of the motor unit appears to be an important factor in SMA genesis and progression which reinforces the potential role of activity-dependent triggering of intracellular signaling such as those dependent on NMDA receptor activation. The deciphering of the intracellular signaling pathways which are activated in the contexts of motor-neuron degeneration or survival permitted the group to understand the importance of a deregulation of the ERK and AKT pathways in the SMA spinal cord and to patent (International patent n°61/489721, 2013) a new therapeutic approach to limit motor-neuron degeneration which is based on the inhibition of the ERK pathway.
Recent Publications
Articles
- Activating ATF6 in spinal muscular atrophy promotes SMN expression and motor neuron survival through the IRE1α-XBP1 pathway.
D’Amico D, Biondi O, Januel C, Bezier C, Sapaly D, Clerc Z, El Khoury M, Sundaram VK, Houdebine L, Josse T, Della Gaspera B, Martinat C, Massaad C, Weill L, Charbonnier F : Neuropathol Appl Neurobiol, 2022 - Small-molecule flunarizine increases SMN protein in nuclear Cajal bodies and motor function in a mouse model of spinal muscular atrophy.
Sapaly D, Dos Santos M, Delers P, Biondi O, Quérol G, Houdebine L, Khoobarry K, Girardet F, Burlet P, Armand AS, Chanoine C, Bureau JF, Charbonnier F, Lefebvre S : Sci Rep, 2018 - Involvement of Aryl hydrocarbon receptor in myelination and in human nerve sheath tumorigenesis.
Shackleford G, Sampathkumar NK, Hichor M, Weill L, Meffre D, Juricek L, Laurendeau I, Chevallier A, Ortonne N, Larousserie F, Herbin M, Bièche I, Coumoul X, Beraneck M, Baulieu EE, Charbonnier F, Pasmant E, Massaad C : Proc Natl Acad Sci U S A, 2018 - Long-term exercise-specific neuroprotection in Spinal Muscular Atrophy-like mice.
Chali F, Desseille C, Houdebine L, Benoit E, Rouquet T, Bariohay B, Lopes P, Branchu J, Gaspera BD, Pariset C, Chanoine C, Charbonnier F, Biondi O : J Physiol, 2016 - Liver X receptors alpha and beta promote myelination and remyelination in the cerebellum.
Meffre D, Shackleford G, Hichor M, Gorgievski V, Tzavara ET, Trousson A, Ghoumari AM, Deboux C, Nait Oumesmar B, Liere P, Schumacher M, Baulieu EE, Charbonnier F, Grenier J, Massaad C : Proc Natl Acad Sci U S A, 2015 - IGF-1R Reduction Triggers Neuroprotective Signaling Pathways in Spinal Muscular Atrophy Mice.
Biondi O, Branchu J, Ben Salah A, Houdebine L, Bertin L, Chali F, Desseille C, Weill L, Sanchez G, Lancelin C, Aïd S, Lopes P, Pariset C, Lécolle S, Côté J, Holzenberger M, Chanoine C, Massaad C, Charbonnier F : J Neurosci, 2015 - Advances in the understanding of skeletal muscle weakness in murine models of diseases affecting nerve-evoked muscle activity, motor neurons, synapses and myofibers.
Ferry A, Joanne P, Hadj-Said W, Vignaud A, Lilienbaum A, Hourdé C, Medja F, Noirez P, Charbonnier F, Chatonnet A, Chevessier F, Nicole S, Agbulut O, Butler-Browne G : Neuromuscul Disord, 2014 - Shift from extracellular signal-regulated kinase to AKT/cAMP response element-binding protein pathway increases survival-motor-neuron expression in spinal-muscular-atrophy-like mice and patient cells.
Branchu J, Biondi O, Chali F, Collin T, Leroy F, Mamchaoui K, Makoukji J, Pariset C, Lopes P, Massaad C, Chanoine C, Charbonnier F : J Neurosci, 2013 - A new role for the calcineurin/NFAT pathway in neonatal myosin heavy chain expression via the NFATc2/MyoD complex during mouse myogenesis.
Daou N, Lecolle S, Lefebvre S, della Gaspera B, Charbonnier F, Chanoine C, Armand AS : Development, 2013 - A novel function for the survival motoneuron protein as a translational regulator.
Sanchez G, Dury AY, Murray LM, Biondi O, Tadesse H, El Fatimy R, Kothary R, Charbonnier F, Khandjian EW, Côté J : Hum Mol Genet, 2012 - Mef2d acts upstream of muscle identity genes and couples lateral myogenesis to dermomyotome formation in Xenopus laevis.
Della Gaspera B, Armand AS, Lecolle S, Charbonnier F, Chanoine C : PLoS One, 2012 - Lithium enhances remyelination of peripheral nerves.
Makoukji J, Belle M, Meffre D, Stassart R, Grenier J, Shackleford G, Fledrich R, Fonte C, Branchu J, Goulard M, de Waele C, Charbonnier F, Sereda MW, Baulieu EE, Schumacher M, Bernard S, Massaad C : Proc Natl Acad Sci U S A, 2012 - Myogenic waves and myogenic programs during Xenopus embryonic myogenesis.
Della Gaspera B, Armand AS, Sequeira I, Chesneau A, Mazabraud A, Lécolle S, Charbonnier F, Chanoine C : Dev Dyn, 2012 - Physical exercise reduces cardiac defects in type 2 spinal muscular atrophy-like mice.
Biondi O, Lopes P, Desseille C, Branchu J, Chali F, Salah AB, Pariset C, Chanoine C, Charbonnier F : J Physiol, 2012 - Mef2d acts
upstream of muscle identity genes and couples lateral myogenesis to dermomyotome
formation in Xenopus laevis.
Della Gaspera B, Armand AS, Lecolle S, Charbonnier F, Chanoine C : PLoS One, 2012 - In vivo NMDA receptor activation accelerates motor unit maturation, protects spinal motor neurons, and enhances SMN2 gene expression in severe spinal muscular atrophy mice.
Biondi O, Branchu J, Sanchez G, Lancelin C, Deforges S, Lopes P, Pariset C, Lécolle S, Côté J, Chanoine C, Charbonnier F : J Neurosci, 2010 - In vivo NMDA receptor activation accelerates motor unit maturation, protects spinal motor neurons, and enhances SMN2 gene expression in severe spinal muscular atrophy mice.
Biondi O, Branchu J, Sanchez G, Lancelin C, Deforges S, Lopes P, Pariset C, Lécolle S, Côté J, Chanoine C, Charbonnier F : J Neurosci, 2010 - The Xenopus MEF2 gene family: evidence of a role for XMEF2C in larval tendon development.
della Gaspera B, Armand AS, Sequeira I, Lecolle S, Gallien CL, Charbonnier F, Chanoine C : Dev Biol, 2009 - Motoneuron survival is promoted by specific exercise in a mouse model of amyotrophic lateral sclerosis.
Deforges S, Branchu J, Biondi O, Grondard C, Pariset C, Lécolle S, Lopes P, Vidal PP, Chanoine C, Charbonnier F : J Physiol, 2009 - Exercise-induced modulation of calcineurin activity parallels the time course of myofibre transitions.
Grondard C, Biondi O, Pariset C, Lopes P, Deforges S, Lécolle S, Gaspera BD, Gallien CL, Chanoine C, Charbonnier F : J Cell Physiol, 2008 - Exercise-induced activation of NMDA receptor promotes motor unit development and survival in a type 2 spinal muscular atrophy model mouse.
Biondi O, Grondard C, Lécolle S, Deforges S, Pariset C, Lopes P, Cifuentes-Diaz C, Li H, della Gaspera B, Chanoine C, Charbonnier F : J Neurosci, 2008 - Exercise-induced neuroprotection in SMA model mice: a means for determining new therapeutic strategies.
Charbonnier F : Mol Neurobiol, 2007 - Sprouty gene expression is regulated by nerve and FGF6 during regeneration of mouse muscles.
Laziz I, Armand AS, Pariset C, Lecolle S, Della Gaspera B, Charbonnier F, Chanoine C : Growth Factors, 2007 - Spatio-temporal expression of MRF4 transcripts and protein during Xenopus laevis embryogenesis.
Della Gaspera B, Sequeira I, Charbonnier F, Becker C, Shi DL, Chanoine C : Dev Dyn, 2006 - FGF6 regulates muscle differentiation through a calcineurin-dependent pathway in regenerating soleus of adult mice.
Armand AS, Pariset C, Laziz I, Launay T, Fiore F, Della Gaspera B, Birnbaum D, Charbonnier F, Chanoine C : J Cell Physiol, 2005 - Regular exercise prolongs survival in a type 2 spinal muscular atrophy model mouse.
Grondard C, Biondi O, Armand AS, Lécolle S, Della Gaspera B, Pariset C, Li H, Gallien CL, Vidal PP, Chanoine C, Charbonnier F : J Neurosci, 2005