Signal pathways and striated muscles
Striated muscles account for about 40% of total body weight, contain 50-75% of the body’s total protein and contribute significantly to multiple body functions. There are two types of striated muscle: skeletal and cardiac muscles. They share a common architecture characterized by a very particular and well described arrangement of muscle cells and associated connective tissues.
Muscular dystrophies correspond to a family of muscle diseases characterized by weakness and progressive muscle degeneration. At the skeletal muscular level, they manifest themselves by a decrease in muscle strength (muscular dystrophy), and a lack of mobilité́ joints (muscle retractions) that begin in childhood or in young adults. The decrease in muscle strength leads, in a few cases, to a loss of independent walking, making it necessary to use a power wheelchair to get around. These are diseases of genetic origin. There are several forms that differ in the age of onset of symptoms, the nature of the muscles affected and their 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 is the main cause of death and makes the severity of these diseases. At present, there is no curative treatment available.
Our group is particularly interested in studying the molecular and cellular mechanisms involved in two muscular dystrophies: Duchenne muscular dystrophy and Emery-Dreifuss muscular dystrophy. It appears important and necessary to increase our knowledge of the pathophysiology of muscular dystrophies and cardiomyopathies in order to unveil the cellular/molecular mechanisms that will allow us to target future therapeutic approaches. We are studying in vitro and in vivo models of these pathologies and developing novel pharmacological therapies based on our discoveries.
Our research is based on 3 axes:
- Tissue organization of striated muscles in health and pathology
- Signalling pathways regulating the links between structure and function in striated muscles
- Control of striated muscle gene expression through signalling pathways
| Name | Position | ORCID |
|---|
- Nicolas Vignier. Le nicotinamide riboside améliore la fonction cardiaque d’un modèle murin de cardiomyopathie dilatée due à des mutations du gène LMNA.. Journée de la societe française de Myologie, Nov 2018, Brest, France. ⟨hal-04012945⟩
- Jean-Claude Kaplan, Antoine Muchir. Glané sur le Web “GLOW”. Médecine/Sciences, 2018, 34, pp.51-51. ⟨10.1051/medsci/201834s217⟩. ⟨hal-02984718⟩
- Astrid Brull, Blanca Morales Rodriguez, Gisele Bonne, Antoine Muchir, Anne T Bertrand. The Pathogenesis and Therapies of Striated Muscle Laminopathies. Frontiers in Physiology, 2018, 9, pp.1533. ⟨10.3389/fphys.2018.01533⟩. ⟨hal-01919521⟩
- Blanca Morales Rodríguez. Exploration of altered molecular pathways involved in pathophysiology of LMNA-cardiomyopathy. Cardiology and cardiovascular system. Sorbonne Université, 2018. English. ⟨NNT : 2018SORUS327⟩. ⟨tel-02864765⟩
- Maria Chatzifrangkeskou, David Yadin, Thibaut Marais, Solenne Chardonnet, Mathilde Cohen-Tannoudji, et al.. Cofilin-1 phosphorylation catalyzed by ERK1/2 alters cardiac actin dynamics in dilated cardiomyopathy caused by lamin A/C gene mutation. Human Molecular Genetics, 2018, 27 (17), pp.3060-3078. ⟨10.1093/hmg/ddy215⟩. ⟨hal-01962065⟩
- Luca Madaro, Magda Passafaro, David Sala, Usue Etxaniz, Francesca Lugarini, et al.. Denervation-activated STAT3–IL-6 signalling in fibro-adipogenic progenitors promotes myofibres atrophy and fibrosis. Nature Cell Biology, 2018, 20 (8), pp.917-927. ⟨10.1038/s41556-018-0151-y⟩. ⟨hal-03859544⟩
- Takuro Arimura, Antoine Muchir, Masayoshi Kuwahara, Sachio Morimoto, Taisuke Ishikawa, et al.. Overexpression of heart-specific small subunit of myosin light chain phosphatase results in heart failure and conduction disturbance. AJP - Heart and Circulatory Physiology, 2018, 314 (6), pp.H1192-H1202. ⟨10.1152/ajpheart.00696.2017⟩. ⟨hal-03859515⟩
- Eleonora Cavallari, Carla Carrera, Matteo Sorge, Gisèle Bonne, Antoine Muchir, et al.. The 13C hyperpolarized pyruvate generated by ParaHydrogen detects the response of the heart to altered metabolism in real time. Scientific Reports, 2018, 8 (1), ⟨10.1038/s41598-018-26583-2⟩. ⟨hal-03270932⟩
- Gabriella Captur, Eloisa Arbustini, Gisèle Bonne, Petros Syrris, Kevin Mills, et al.. Lamin and the heart. Heart, 2018, 104 (6), pp.468-479. ⟨10.1136/heartjnl-2017-312338⟩. ⟨hal-03285171⟩
- Nicolas Vignier, Maria Chatzifrangkeskou, Blanca Morales Rodriguez, Mathias Mericskay, Nathalie Mougenot, et al.. Rescue of biosynthesis of nicotinamide adenine dinucleotide protects the heart in cardiomyopathy caused by lamin A/C gene mutation. Human Molecular Genetics, 2018, 27 (22), pp.3870-3880. ⟨10.1093/hmg/ddy278⟩. ⟨hal-01958003⟩
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