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 |
|---|
- Francesco Girardi. TGFbeta signalling pathway in muscle regeneration : an important regulator of muscle cell fusion. Cellular Biology. Sorbonne Université, 2019. English. ⟨NNT : 2019SORUS114⟩. ⟨tel-02944744⟩
- Nicolas Vignier, Nathalie Mougenot, Gisèle Bonne, Antoine Muchir. Effect of genetic background on the cardiac phenotype in a mouse model of Emery-Dreifuss muscular dystrophy. Biochemistry and Biophysics Reports, 2019, 19, pp.100664. ⟨10.1016/j.bbrep.2019.100664⟩. ⟨hal-03464625⟩
- Kun Wang, Lea Trichet, Clément Rieu, Cécile Peccate, Gaëlle Pembouong, et al.. Interactions of Organosilanes with Fibrinogen and Their Influence on Muscle Cell Proliferation in 3D Fibrin Hydrogels. Biomacromolecules, 2019, ⟨10.1021/acs.biomac.9b00686⟩. ⟨hal-02274878⟩
- Karim Wahbi, Rabah Ben Yaou, Estelle Gandjbakhch, Frédéric Anselme, Thomas Gossios, et al.. Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies. Circulation, 2019, 140 (4), pp.293-302. ⟨10.1161/CIRCULATIONAHA.118.039410⟩. ⟨hal-02237297⟩
- Lorenzo Giordani, Gary He, Elisa Negroni, Hiroshi Sakai, Justin Y.C. Law, et al.. High-Dimensional Single-Cell Cartography Reveals Novel Skeletal Muscle-Resident Cell Populations. Molecular Cell, 2019, 74 (3), pp.609-621.e6. ⟨10.1016/j.molcel.2019.02.026⟩. ⟨hal-02376541⟩
- Nicolas Vignier. Transcriptomic analysis of soleus muscle from a mouse model of Emery-Dreifuss muscular dystrophy. MYOLOGY, Mar 2019, Bardeaux, France. ⟨hal-04012925⟩
- Rémi Thomasson, Nicolas Vignier, Cécile Peccate, Nathalie Mougenot, Philippe Noirez, et al.. Alteration of performance in a mouse model of Emery–Dreifuss muscular dystrophy caused by A-type lamins gene mutation. Human Molecular Genetics, 2019, 28 (13), pp.2237-2244. ⟨10.1093/hmg/ddz056⟩. ⟨hal-02404836⟩
- Antoine Muchir. Cytoskeleton and conduction defects. Conference, GRRC, 2019, Lille, France. ⟨hal-04000841⟩
- Antoine Muchir. Altered cytoskeleton in cardiac disease caused by nuclear A-type lamins gene mutations. Conference, Myology, 2019, Bordeaux, France. ⟨hal-04000844⟩
- Barbora Malecova, Sole Gatto, Usue Etxaniz, Magda Passafaro, Amy Cortez, et al.. Dynamics of cellular states of fibro-adipogenic progenitors during myogenesis and muscular dystrophy. Nature Communications, 2018, 9 (1), pp.3670. ⟨10.1038/s41467-018-06068-6⟩. ⟨hal-03859529⟩
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