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Excitabilité et signalisation calcique dans le muscle squelettique normal et malade

Vincent Jacquemond

À propos

Nos recherches sont axées sur la physiologie et la physiopathologie de la fonction musculaire squelettique. Elle vise à comprendre comment les mécanismes impliqués dans le contrôle du Ca²⁺+ L’homéostasie musculaire et le couplage excitation-contraction (EC) fonctionnent dans des conditions normales et pathologiques. Pour ce faire, nous utilisons une combinaison de biologie moléculaire, de biochimie, de transfert de gènes in vivo et de détection simultanée d’électrophysiologie et de fluorescence sur des cellules cultivées et sur des fibres musculaires différenciées isolées.

La contraction musculaire est initiée lorsque des potentiels d’action déclenchés à la plaque terminale des cellules musculaires se propagent à travers la membrane plasmique et déclenchent un changement de conformation de la protéine Ca_V1.1 qui ouvre un canal Ca²⁺(récepteur de la ryanodine de type 1, RyR1) dans la membrane du réticulum sarcoplasmique (SR). Le Ca²⁺est alors libéré du SR dans le cytosol et déclenche la contraction. Outre le Ca²⁺de la SR, il y a également une libération de Ca²⁺à partir du milieu extracellulaire.

Le projet global repose sur un ensemble de méthodes et d’expertises comprenant la biologie moléculaire et la biochimie, le transfert de gènes in vivo et une combinaison de pointe d’électrophysiologie et de détection de fluorescence sur des cellules musculaires différenciées isolées de la souris.

Membres

Vincent Jacquemond

Christine Berthier

Professeur assistant

Stephan Collins

Professeur assistant

Camille Dauvois

Assistant ingénieur

Céline Malleval

Jonathan Schreiber

Emilia Skutunova

Chercheur principal

Evénements

men and women sitting on chairs inside room

Étude de la fonction du réticulum sarcoplasmique pendant le couplage excitation-contraction du muscle squelettique à l’aide de biocapteurs fluorescents

Colline Sanchez

Le couplage excitation-contraction (EC) dans le muscle squelettique correspond à la séquence d’événements par laquelle la contraction de la fibre […]

Actualités

Distinction spéciale pour l’article de Bruno Allard dans The American Physiological Society

Bruno Allard’s 2018 article received a special distinction from The American Physiological Society.

Projets

Comprendre les mécanismes de base impliqués dans la régulation du CaV1.1 et de la fonction RyR1

Responsable Vincent Jacquemond

Équipe: Excitabilité et signalisation calcique dans le muscle squelettique normal et malade

Mutations pathologiques perturbant l’excitabilité et le couplage EC dans les muscles squelettiques

Responsable Vincent Jacquemond

Équipe: Excitabilité et signalisation calcique dans le muscle squelettique normal et malade

Publications

Jaque-Fernandez F, Demesmay L, Idoux R, Berthier C, Jacquemond V, Allard B, Mn quenching in activated zebrafish muscle fibers does not result from store-operated Ca entry., J Gen Physiol 2026 Jan; 158(1): .

Demesmay L, Idoux R, Berthier C, Bernat C, Espinosa L, Jacquemond V, Brunet F, Maunier-Mercier A, Lo..., Zebrafish fast muscle contractions avoid the mammalian requirement for voltage-gated Na+ channels., PLoS Biol 2025 Nov; 23(11): e3003484.

Melka M, Rotard L, Benstaali C, Brocard J, Giannesini B, Jouve F, Pelletier L, Fauré J, Rendu J, Ja..., Limited pre-clinical relevance of the heterozygous RYR1-I4895T/+ mouse model due to its mild phenotype., J Neuromuscul Dis 2025 Sep; (): 22143602251339354.

Guiraud A, Couturier N, Christin E, Castellano L, Daura M, Kretz-Remy C, Janin A, Ghasemizadeh A, De..., SH3KBP1 promotes skeletal myofiber formation and functionality through ER/SR architecture integrity., EMBO Rep 2025 Apr; 26(8): 2166-2191.

Schreiber J, Rotard L, Tourneur Y, Lafoux A, Berthier C, Allard B, Huchet C, Jacquemond V, Reduced voltage-activated Ca2+ release flux in muscle fibers from a rat model of Duchenne dystrophy., J Gen Physiol 2025 Mar; 157(2): .

Flück M, Sanchez C, Jacquemond V, Berthier C, Giraud MN, Jacko D, Bersiner K, Gehlert S, Baan G, Ja..., Enhanced capacity for CaMKII signaling mitigates calcium release related contractile fatigue with high intensity exercise., Biochim Biophys Acta Mol Cell Res 2024 Feb; 1871(2): 119610.

Idoux R, Exbrayat-Héritier C, Sohm F, Jaque-Fernandez F, Vaganay E, Berthier C, Bretaud S, Jacquemo..., A mechano- and heat-gated two-pore domain K+ channel controls excitability in adult zebrafish skeletal muscle., Proc Natl Acad Sci U S A 2023 Nov; 120(45): e2305959120.

Jaque-Fernandez F, Allard B, Monteiro L, Lafoux A, Huchet C, Jaimovich E, Berthier C, Jacquemond V, Probenecid affects muscle Ca2+ homeostasis and contraction independently from pannexin channel block., J Gen Physiol 2023 Apr; 155(4): .

Cikes D, Elsayad K, Sezgin E, Koitai E, Torma F, Orthofer M, Yarwood R, Heinz LX, Sedlyarov V, Miran..., Author Correction: PCYT2-regulated lipid biosynthesis is critical to muscle health and ageing., Nat Metab 2023 Apr; 5(4): 711.

Cikes D, Elsayad K, Sezgin E, Koitai E, Torma F, Orthofer M, Yarwood R, Heinz LX, Sedlyarov V, Miran..., PCYT2-regulated lipid biosynthesis is critical to muscle health and ageing., Nat Metab 2023 Mar; 5(3): 495-515.

Svahn J, Coudert L, Streichenberger N, Kraut A, Gravier-Dumonceau-Mazelier A, Rotard L, Calemard-Mic..., Immune-Mediated Rippling Muscle Disease Associated With Thymoma and Anti-MURC/Cavin-4 Autoantibodies., Neurol Neuroimmunol Neuroinflamm 2023 Jan; 10(1): .

Szentesi P, Dienes B, Kutchukian C, Czirjak T, Buj-Bello A, Jacquemond V, Csernoch L, Disrupted T-tubular network accounts for asynchronous calcium release in MTM1-deficient skeletal muscle., J Physiol 2023 Jan; 601(1): 99-121.

Idoux R, Bretaud S, Berthier C, Ruggiero F, Jacquemond V, Allard B, Superfast excitation-contraction coupling in adult zebrafish skeletal muscle fibers., J Gen Physiol 2022 Sep; 154(9): .

Silva-Rojas R, Nattarayan V, Jaque-Fernandez F, Gomez-Oca R, Menuet A, Reiss D, Goret M, Messaddeq N..., Mice with muscle-specific deletion of Bin1 recapitulate centronuclear myopathy and acute downregulation of dynamin 2 improves their phenotypes., Mol Ther 2022 Feb; 30(2): 868-880.

Jaque-Fernández F, Jorquera G, Troc-Gajardo J, Pietri-Rouxel F, Gentil C, Buvinic S, Allard B, Jaim..., Pannexin-1 and CaV1.1 show reciprocal interaction during excitation-contraction and excitation-transcription coupling in skeletal muscle., J Gen Physiol 2021 Dec; 153(12): .

Ghasemizadeh A, Christin E, Guiraud A, Couturier N, Abitbol M, Risson V, Girard E, Jagla C, Soler C,..., MACF1 controls skeletal muscle function through the microtubule-dependent localization of extra-synaptic myonuclei and mitochondria biogenesis., Elife 2021 Aug; 10(): .

Sanchez C, Berthier C, Tourneur Y, Monteiro L, Allard B, Csernoch L, Jacquemond V, Detection of Ca2+ transients near ryanodine receptors by targeting fluorescent Ca2+ sensors to the triad., J Gen Physiol 2021 Apr; 153(4): .

Prokic I, Cowling BS, Kutchukian C, Kretz C, Tasfaout H, Gache V, Hergueux J, Wendling O, Ferry A, T..., Differential physiological roles for BIN1 isoforms in skeletal muscle development, function and regeneration., Dis Model Mech 2020 Nov; 13(11): .

Pelletier L, Petiot A, Brocard J, Giannesini B, Giovannini D, Sanchez C, Travard L, Chivet M, Beaufi..., In vivo RyR1 reduction in muscle triggers a core-like myopathy., Acta Neuropathol Commun 2020 Nov; 8(1): 192.

Jaque-Fernandez F, Beaulant A, Berthier C, Monteiro L, Allard B, Casas M, Rieusset J, Jacquemond V, Preserved Ca2+ handling and excitation-contraction coupling in muscle fibres from diet-induced obese mice., Diabetologia 2020 Nov; 63(11): 2471-2481.

Idoux R, Fuster C, Jacquemond V, Dayal A, Grabner M, Charnet P, Allard B, Divalent cations permeation in a Ca2+ non-conducting skeletal muscle dihydropyridine receptor mouse model., Cell Calcium 2020 Nov; 91(): 102256.

Idoux R, Bretaud S, Berthier C, Jacquemond V, Ruggiero F, Allard B, [Unraveling the pathophysiology of Bethlem Myopathy using a unique zebrafish model for the disease]., Med Sci (Paris) 2019 Nov; 35 Hors série n° 2(): 39-42.

Rossi D, Scarcella AM, Liguori E, Lorenzini S, Pierantozzi E, Kutchukian C, Jacquemond V, Messa M, D..., Molecular determinants of homo- and heteromeric interactions of Junctophilin-1 at triads in adult skeletal muscle fibers., Proc Natl Acad Sci U S A 2019 Jul; 116(31): 15716-15724.

Kutchukian C, Szentesi P, Allard B, Buj-Bello A, Csernoch L, Jacquemond V, Ca2+-induced sarcoplasmic reticulum Ca2+ release in myotubularin-deficient muscle fibers., Cell Calcium 2019 Jun; 80(): 91-100.

Alireza Ghasemizadeh, Emilie Christin, Alexandre Guiraud, Nathalie Couturier, Valérie Risson, Emman..., Skeletal muscle MACF1 maintains myonuclei and mitochondria localization through microtubules to control muscle functionalities, .

Fuster C, Idoux R, Berthier C, Jacquemond V, Allard B, Mammalian skeletal muscle does not express functional voltage-gated H+ channels., Am J Physiol Cell Physiol 2018 Nov; 315(5): C776-C779.

Sanchez C, Berthier C, Allard B, Perrot J, Bouvard C, Tsutsui H, Okamura Y, Jacquemond V, Tracking the sarcoplasmic reticulum membrane voltage in muscle with a FRET biosensor., J Gen Physiol 2018 Aug; 150(8): 1163-1177.

Huntoon V, Widrick JJ, Sanchez C, Rosen SM, Kutchukian C, Cao S, Pierson CR, Liu X, Perrella MA, Beg..., SPEG-deficient skeletal muscles exhibit abnormal triad and defective calcium handling., Hum Mol Genet 2018 May; 27(9): 1608-1617.

Kutchukian C, Szentesi P, Allard B, Trochet D, Beuvin M, Berthier C, Tourneur Y, Guicheney P, Cserno..., Impaired excitation-contraction coupling in muscle fibres from the dynamin2R465W mouse model of centronuclear myopathy., J Physiol 2017 Dec; 595(24): 7369-7382.

Fuster C, Perrot J, Berthier C, Jacquemond V, Charnet P, Allard B, Na leak with gating pore properties in hypokalemic periodic paralysis V876E mutant muscle Ca channel., J Gen Physiol 2017 Dec; 149(12): 1139-1148.

Fuster C, Perrot J, Berthier C, Jacquemond V, Allard B, Elevated resting H+ current in the R1239H type 1 hypokalaemic periodic paralysis mutated Ca2+ channel., J Physiol 2017 Oct; 595(20): 6417-6428.

Kutchukian C, Lo Scrudato M, Tourneur Y, Poulard K, Vignaud A, Berthier C, Allard B, Lawlor MW, Buj-..., Phosphatidylinositol 3-kinase inhibition restores Ca2+ release defects and prolongs survival in myotubularin-deficient mice., Proc Natl Acad Sci U S A 2016 Dec; 113(50): 14432-14437.

Bodnár D, Csernoch L, Jacquemond V, Maurocalcin phosphorylated at threonin 26 maintains its activity on ryanodine receptor-mediated Ca2+ release in intact muscle fibers., Proc Natl Acad Sci U S A 2016 Jul; 113(30): E4264-5.

Csernoch L, Jacquemond V, Phosphoinositides in Ca(2+) signaling and excitation-contraction coupling in skeletal muscle: an old player and newcomers., J Muscle Res Cell Motil 2015 Dec; 36(6): 491-9.

Berthier C, Kutchukian C, Bouvard C, Okamura Y, Jacquemond V, Depression of voltage-activated Ca2+ release in skeletal muscle by activation of a voltage-sensing phosphatase., J Gen Physiol 2015 Apr; 145(4): 315-30.

Rodríguez EG, Lefebvre R, Bodnár D, Legrand C, Szentesi P, Vincze J, Poulard K, Bertrand-Michel J,..., Phosphoinositide substrates of myotubularin affect voltage-activated Ca²⁺ release in skeletal muscle., Pflugers Arch 2014 May; 466(5): 973-85.

Lefebvre R, Pouvreau S, Collet C, Allard B, Jacquemond V, Whole-cell voltage clamp on skeletal muscle fibers with the silicone-clamp technique., Methods Mol Biol 2014 ; 1183(): 159-70.

Jorquera G, Altamirano F, Contreras-Ferrat A, Almarza G, Buvinic S, Jacquemond V, Jaimovich E, Casas..., Cav1.1 controls frequency-dependent events regulating adult skeletal muscle plasticity., J Cell Sci 2013 Mar; 126(Pt 5): 1189-98.

Lefebvre R, Legrand C, Groom L, Dirksen RT, Jacquemond V, Ca2+ release in muscle fibers expressing R4892W and G4896V type 1 ryanodine receptor disease mutants., PLoS One 2013 ; 8(1): e54042.

Jacquemond V, Waveless mammalian muscle., J Physiol 2012 Apr; 590(8): 1783.

Golini L, Chouabe C, Berthier C, Cusimano V, Fornaro M, Bonvallet R, Formoso L, Giacomello E, Jacque..., Junctophilin 1 and 2 proteins interact with the L-type Ca2+ channel dihydropyridine receptors (DHPRs) in skeletal muscle., J Biol Chem 2011 Dec; 286(51): 43717-43725.

Lefebvre R, Legrand C, González-Rodríguez E, Groom L, Dirksen RT, Jacquemond V, Defects in Ca2+ release associated with local expression of pathological ryanodine receptors in mouse muscle fibres., J Physiol 2011 Nov; 589(Pt 22): 5361-82.

Weiss N, Legrand C, Pouvreau S, Bichraoui H, Allard B, Zamponi GW, De Waard M, Jacquemond V, In vivo expression of G-protein beta1gamma2 dimer in adult mouse skeletal muscle alters L-type calcium current and excitation-contraction coupling., J Physiol 2010 Aug; 588(Pt 15): 2945-60.

Weiss N, Andrianjafiniony T, Dupré-Aucouturier S, Pouvreau S, Desplanches D, Jacquemond V, Altered myoplasmic Ca(2+) handling in rat fast-twitch skeletal muscle fibres during disuse atrophy., Pflugers Arch 2010 Mar; 459(4): 631-44.

Al-Qusairi L, Weiss N, Toussaint A, Berbey C, Messaddeq N, Kretz C, Sanoudou D, Beggs AH, Allard B, ..., T-tubule disorganization and defective excitation-contraction coupling in muscle fibers lacking myotubularin lipid phosphatase., Proc Natl Acad Sci U S A 2009 Nov; 106(44): 18763-8.

Weiss N, Couchoux H, Legrand C, Berthier C, Allard B, Jacquemond V, Expression of the muscular dystrophy-associated caveolin-3(P104L) mutant in adult mouse skeletal muscle specifically alters the Ca(2+) channel function of the dihydropyridine receptor., Pflugers Arch 2008 Nov; 457(2): 361-75.

Ram N, Weiss N, Texier-Nogues I, Aroui S, Andreotti N, Pirollet F, Ronjat M, Sabatier JM, Darbon H, ..., Design of a disulfide-less, pharmacologically inert, and chemically competent analog of maurocalcine for the efficient transport of impermeant compounds into cells., J Biol Chem 2008 Oct; 283(40): 27048-56.

Legrand C, Giacomello E, Berthier C, Allard B, Sorrentino V, Jacquemond V, Spontaneous and voltage-activated Ca2+ release in adult mouse skeletal muscle fibres expressing the type 3 ryanodine receptor., J Physiol 2008 Jan; 586(2): 441-57.

Csernoch L, Pouvreau S, Ronjat M, Jacquemond V, Voltage-activated elementary calcium release events in isolated mouse skeletal muscle fibers., J Membr Biol 2008 ; 226(1-3): 43-55.

Couchoux H, Allard B, Legrand C, Jacquemond V, Berthier C, Loss of caveolin-3 induced by the dystrophy-associated P104L mutation impairs L-type calcium channel function in mouse skeletal muscle cells., J Physiol 2007 May; 580(Pt.3): 745-54.

Pouvreau S, Collet C, Allard B, Jacquemond V, Whole-cell voltage clamp on skeletal muscle fibers with the silicone-clamp technique., Methods Mol Biol 2007 ; 403(): 185-94.

Pouvreau S, Csernoch L, Allard B, Sabatier JM, De Waard M, Ronjat M, Jacquemond V, Transient loss of voltage control of Ca2+ release in the presence of maurocalcine in skeletal muscle., Biophys J 2006 Sep; 91(6): 2206-15.

Allard B, Couchoux H, Pouvreau S, Jacquemond V, Sarcoplasmic reticulum Ca2+ release and depletion fail to affect sarcolemmal ion channel activity in mouse skeletal muscle., J Physiol 2006 Aug; 575(Pt 1): 69-81.

Pouvreau S, Jacquemond V, Nitric oxide synthase inhibition affects sarcoplasmic reticulum Ca2+ release in skeletal muscle fibres from mouse., J Physiol 2005 Sep; 567(Pt 3): 815-28.

Pouvreau S, Allard B, Berthier C, Jacquemond V, Control of intracellular calcium in the presence of nitric oxide donors in isolated skeletal muscle fibres from mouse., J Physiol 2004 Nov; 560(Pt 3): 779-94.

Collet C, Pouvreau S, Csernoch L, Allard B, Jacquemond V, Calcium signaling in isolated skeletal muscle fibers investigated under “Silicone Voltage-Clamp” conditions., Cell Biochem Biophys 2004 ; 40(2): 225-36.

Jospin M, Jacquemond V, Mariol MC, Ségalat L, Allard B, The L-type voltage-dependent Ca2+ channel EGL-19 controls body wall muscle function in Caenorhabditis elegans., J Cell Biol 2002 Oct; 159(2): 337-48.

Collet C, Jacquemond V, Sustained release of calcium elicited by membrane depolarization in ryanodine-injected mouse skeletal muscle fibers., Biophys J 2002 Mar; 82(3): 1509-23.

Collet C, Strube C, Csernoch L, Mallouk N, Ojeda C, Allard B, Jacquemond V, Effects of extracellular ATP on freshly isolated mouse skeletal muscle cells during pre-natal and post-natal development., Pflugers Arch 2002 Mar; 443(5-6): 771-8.