Myotube elasticity of an amyotrophic lateral sclerosis mouse model
| Autor: | Frédéric Cuisinier, Marta Martin-Fernandez, Ana Sanchez-Vicente, Céline Salsac, Béla Varga, Csilla Gergely, Cédric Raoul, Frédérique Scamps, Cécile Hilaire, Julie Areias |
|---|---|
| Přispěvatelé: | Gergely, Csilla, Laboratoire Charles Coulomb (L2C), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Laboratoire de Bioingénierie et NanoSciences (LBN), Université de Montpellier (UM), Institut des Neurosciences de Montpellier (INM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Université Montpellier 1 (UM1)-Université de Montpellier (UM), Institut des Neurosciences de Montpellier - Déficits sensoriels et moteurs (INM) |
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
[PHYS.PHYS.PHYS-BIO-PH]Physics [physics]/Physics [physics]/Biological Physics [physics.bio-ph] Muscle Fibers Skeletal SOD1 Population lcsh:Medicine Myosins Biology Microscopy Atomic Force Muscle Development Article Mice 03 medical and health sciences Superoxide Dismutase-1 0302 clinical medicine Myosin medicine Animals Humans Amyotrophic lateral sclerosis education lcsh:Science Actin Mechanical Phenomena education.field_of_study Muscle Weakness Multidisciplinary [PHYS.PHYS.PHYS-BIO-PH] Physics [physics]/Physics [physics]/Biological Physics [physics.bio-ph] Myogenesis Amyotrophic Lateral Sclerosis lcsh:R Muscle weakness Skeletal muscle Cell Differentiation medicine.disease Actins Elasticity Cell biology Disease Models Animal 030104 developmental biology medicine.anatomical_structure Gene Expression Regulation Mutation lcsh:Q medicine.symptom 030217 neurology & neurosurgery |
| Zdroj: | Scientific Reports, Vol 8, Iss 1, Pp 1-10 (2018) Scientific Reports Scientific Reports, 2018, ⟨10.1038/s41598-018-24027-5⟩ Scientific Reports, Nature Publishing Group, 2018, ⟨10.1038/s41598-018-24027-5⟩ |
| ISSN: | 2045-2322 |
| DOI: | 10.1038/s41598-018-24027-5 |
| Popis: | Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects the motor system leading to generalized paralysis and death of patients. The understanding of early pathogenic mechanisms will help to define early diagnostics criteria that will eventually provide basis for efficient therapeutics. Early symptoms of ALS usually include muscle weakness or stiffness. Therefore, mechanical response of differentiated myotubes from primary cultures of mice, expressing the ALS-causing SOD1 G93A mutation, was examined by atomic force microscopy. Simultaneous acquisition of topography and cell elasticity of ALS myotubes was performed by force mapping method, compared with healthy myotubes and supplemented with immunofluorescence and qRT-PCR studies. Wild type myotubes reveal a significant difference in elasticity between a narrow and a wide population, consistent with maturation occurring with higher actin expression relative to myosin together with larger myotube width. However, this is not true for SOD1 G93A expressing myotubes, where a significant shift of thin population towards higher elastic modulus values was observed. We provide evidence that SOD1 mutant induces structural changes that occurs very early in muscle development and well before symptomatic stage of the disease. These findings could significantly contribute to the understanding of the role of skeletal muscle in ALS pathogenesis. |
| Databáze: | OpenAIRE |
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