
Team leader: Antoine Muchir, PhD
Striated muscles comprise approximately 40% of total body weight, contain 50–75% of all body proteins and contribute significantly to multiple bodily functions. Two types of striated muscles exist: skeletal and cardiac muscles. They share a common architecture characterized by a very particular and well-described arrangement of muscle cells and associated connective tissue.
Muscular dystrophies are a family of muscle diseases characterized by weakness and progressive muscle degeneration. At the skeletal muscle level, they are manifested by a decrease of muscle strength (muscular dystrophy), and a lack of mobility of the joints (muscle retractions) that begin in childhood or in young adults. In a few cases, the decrease in muscle strength results in a loss of independent walking, making it necessary to use an electric wheelchair to move. These are genetically inherited diseases. There are several forms that differ in the age of onset of symptoms, the nature of the affected muscles, and severity. At the cardiac level, the presence of abnormalities is observed at a more or less advanced age, mainly in the form of dilated cardiomyopathy which represents the main cause of death and the severity of these diseases. There is still no cure so far.
Our group is particularly interested in studying the molecular and cellular mechanisms involved in muscular dystrophies and cardiomyopathies. It seems important and necessary to increase our knowledge on the pathophysiology of these pathologies to unveil the cellular / molecular mechanisms that will make it possible to target future therapeutic approaches. We study in vitro and in vivo models of these pathologies and we develop new pharmacological therapies based on our findings.

AIM Equipe 9 – Voies de signalisation & muscles striés dirigée par Antoine Muchir le 30 avril 2024
Team members
Antoine MUCHIR, PhD, DR2, Team Leader
(google scholar : https://scholar.google.fr/citations?user=o2t5Nr0AAAAJ&hl=fr)
(ORCID : https://orcid.org/0000-0002-4780-9275)
Nicolas VIGNIER, PhD, CR
Bruno CADOT, PhD, CR
Cécile PECATTE, IE
Stéphanie BAUCHÉ, PhD, IR
Lorenzo GIORDANI, PhD, CRCN
Anne FORAND, PhD, Researcher
Elisabeth EUPHROSINE, PhD student
Chiara D’ERCOLE, PhD, Post-doc
Lucile SAILLARD, Ingénieure d’Études
Pritam SAHA, PhD student
Joana DA COSTA ORTERELO MARTINS, PhD student
Zoheir GUESMIA, IE (20%)
Last publications
- Forand A, Moog S, Mougenot N, Lemaitre M, Sevoz-Couche C, Guesmia Z, Virtanen L, Giordani L, Muchir A, Pietri-Rouxel F. Long-Term Dystrophin Replacement Therapy in Duchenne Muscular Dystrophy Causes Cardiac Inflammation. JACC Basic Transl Sci. 2025;10(6):759-782.
- Ruggieri V, Scaricamazza S, Bracaglia A, D’Ercole C, Parisi C, D’Angelo P, Proietti D, Cappelletti C, Macone A, Lozanoska-Ochser B, Bouchè M, Latella L, Valle C, Ferri A, Giordani L, Madaro L. Polyamine metabolism dysregulation contributes to muscle fiber vulnerability in ALS. Cell Rep. 2025;44(1):115123.
- Chassagne J, Da Silva N, Akrouf I, Cadot B, Julien L, Barthélémy I, Blot S, Le Guiner C, Bui MT, Romero NB, Lainé J, Pietri-Rouxel F, Meunier P, Mamchaoui K, Lorain S, Bitoun M, Benkhelifa-Ziyyat S. Early endosome disturbance and endolysosomal pathway dysfunction in Duchenne muscular dystrophy. Am J Pathol. 2025:S0002-9440(25)00189-0.
- Cardoso D, Guilbert S, Guigue P, Carabalona A, Harhouri K, Peccate C, Tournois J, Guesmia Z, Ferreira L, Bartoli C, Levy N, Colleaux L, Nissan X, Muchir A. Inhibition of poly(ADP-Ribosyl)ation reduced vascular smooth muscle cells loss and improves aortic disease in a mouse model of human accelerated aging syndrome. Cell Death Dis. 2024;15(10):723.
- Garcia P, Jarassier W, Brun C, Giordani L, Agostini F, Kung WH, Peccate C, Ravent J, Fall S, Petit V, Cheung TH, Ait-Si-Ali S, Le Grand F. Setdb1 protects genome integrity in murine muscle stem cells to allow for regenerative myogenesis and inflammation. Dev Cell. 2024; 59(17):2375-2392.
- Traoré M, Noviello C, Vergnol A, Gentil C, Halliez M, Saillard L, Gelin M, Forand A, Lemaitre M, Guesmia Z, Cadot B, Caldas de Almeida Araujo E, Marty B, Mougenot N, Messéant J, Strochlic L, Sadoine J, Slimani L, Jolly A, De la Grange P, Hogrel JY, Pietri-Rouxel F, Falcone S. GDF5 as a rejuvenating treatment for age-related neuromuscular failure. Brain. 2024;147(11):3834-3848.
- Ferrand MC, Giordano G, Mougenot N, Laporte PL, Vignier N, Leclerc A, Algalarrondo V, Extramiana F, Charpentier F, Neyroud N. Intracardiac electrophysiology to characterize susceptibility to ventricular arrhythmias in murine models. Front Physiol. 2024:15:1326663.
- Rose N, Chavez BE, Sonam S, Nguyen T, Grenci G, Bigot A, Muchir A, Ladoux B, Cadot B, Le Grand F, Trichet L. Bioengineering a miniaturized in vitro 3D myotube contraction monitoring chip to model muscular dystrophies. Biomaterials. 2023;293:121935. doi:10.1016/j.biomaterials.2022.121935
- Chatzifrangkeskou M, Le Dour C, Muchir A. Modulation of cytoskeleton in cardiomyopathy caused by mutations in LMNA gene. AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY. 2023;324(6):C1223-C1235. doi:10.1152/ajpcell.00471.2022
- Le Dour C, Chatzifrangkeskou M, Macquart C, Magiera MM, Peccate C, Jouve C, Virtanen L, Heliö T, Aalto-Setälä K, Crasto S, Cadot B, Cardoso D, Mougenot N, Adesse D, Di Pasquale E, Hulot JS, Taimen P, Janke C, Muchir A. Actin-microtubule cytoskeletal interplay mediated by MRTF-A/SRF signaling promotes dilated cardiomyopathy caused by LMNA mutations. Nat Commun. 2022 Dec 22;13(1):7886. doi: 10.1038/s41467-022-35639-x. PMID: 36550158
- Kervella M, Jahier M, Meli AC, Muchir A. Genome organization in cardiomyocytes expressing mutated A-type lamins. Front Cell Dev Biol. 2022 Oct 7;10:1030950. doi: 10.3389/fcell.2022.1030950. eCollection 2022. PMID: 36274847