Mutation in the Core Structure of Desmin Intermediate Filaments Affects Myoblast Elasticity
| Autor: | Fatma Briki, Florence Delort, Felix Rico, Abel de Sousa Moreira, Sabrina Batonnet-Pichon, C. Even, Patrick Vicart, Virginie Bailleux, Anna F. Rigato, G. Abramovici |
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| Přispěvatelé: | Laboratoire de Physique des Solides (LPS), Université Paris-Sud - Paris 11 (UP11)-Centre National de la Recherche Scientifique (CNRS), Unité de Biologie Fonctionnelle et Adaptative (BFA (UMR_8251 / U1133)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Physics Department-UFR925, Université Pierre et Marie Curie - Paris 6 (UPMC), BIO-AFM-LAB Bio Atomic Force Microscopy Laboratory (Bio-AFM-Lab), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris-Sud - Paris 11 (UP11), Centre National de la Recherche Scientifique (CNRS)-Université Paris-Sud - Paris 11 (UP11), Laboratoire de Physique des Solides ( LPS ), Université Paris-Sud - Paris 11 ( UP11 ) -Centre National de la Recherche Scientifique ( CNRS ), Biologie Fonctionnelle et Adaptative ( BFA ), Université Paris Diderot - Paris 7 ( UPD7 ) -Centre National de la Recherche Scientifique ( CNRS ), Université Pierre et Marie Curie - Paris 6 ( UPMC ), Laboratoire de Physique des Solides (UMR CNRS 8502 - Université Paris-Sud) ( LPS ), BIO-AFM-LAB Bio Atomic Force Microscopy Laboratory ( Bio-AFM-Lab ), Aix Marseille Université ( AMU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Université Paris-Sud - Paris 11 ( UP11 ) |
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
[SDV]Life Sciences [q-bio] Biophysics Intermediate Filaments 02 engineering and technology macromolecular substances Protein aggregation Cell Line Desmin Myoblasts 03 medical and health sciences Protein Aggregates Protein Domains Fluorescence microscope Myocyte Humans Intermediate filament Elastic modulus [ SDV ] Life Sciences [q-bio] Chemistry 021001 nanoscience & nanotechnology Actin cytoskeleton musculoskeletal system Elasticity Crystallography 030104 developmental biology Cell Biophysics Mutation 0210 nano-technology Myofibril |
| Zdroj: | Biophysical Journal Biophysical Journal, 2017, 113 (3), pp.627-636. ⟨10.1016/j.bpj.2017.06.020⟩ Biophysical Journal, Biophysical Society, 2017, 113 (3), pp.627-636. ⟨10.1016/j.bpj.2017.06.020⟩ Biophysical Journal, Biophysical Society, 2017, 113 (3), pp.627-636. 〈10.1016/j.bpj.2017.06.020〉 |
| ISSN: | 1542-0086 0006-3495 |
| DOI: | 10.1016/j.bpj.2017.06.020⟩ |
| Popis: | International audience; Elastic properties of cells are mainly derived from the actin cytoskeleton. However, intermediate filaments are emerging as major contributors to the mechanical properties of cells. Using atomic force microscopy, we studied the elasticity of mouse myoblasts expressing a mutant form of the gene encoding for desmin intermediate filaments, p.D399Y. This variant produces desmin aggregates, the main pathological symptom of myofibrillar myopathies. Here we show that desmin-mutated cells display a 39% increased median elastic modulus compared to wild-type cells. Desmin-mutated cells required higher forces than wild-type cells to reach high indentation depths, where desmin intermediate filaments are typically located. In addition, heat shock treatment increased the proportion of cells with aggregates and induced a secondary peak in the distribution of Young's. moduli. By performing atomic force microscopy mechanical mapping combined with fluorescence microscopy, we show that higher Young's moduli were measured where desmin aggregates were located, indicating that desmin aggregates are rigid. Therefore, we provide evidence that p.D399Y stiffens mouse myoblasts. Based on these results, we suggest that p.D399Y-related myofibrillar myopathy is at least partly due to altered mechanical properties at the single-cell scale, which are propagated to the tissue scale. |
| Databáze: | OpenAIRE |
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