Desmin and Plectin Recruitment to the Nucleus and Nuclei Orientation Are Lost in Emery-Dreifuss Muscular Dystrophy Myoblasts Subjected to Mechanical Stimulation

Author:

Cenni Vittoria12ORCID,Evangelisti Camilla3ORCID,Santi Spartaco12ORCID,Sabatelli Patrizia12,Neri Simona4ORCID,Cavallo Marco5ORCID,Lattanzi Giovanna12ORCID,Mattioli Elisabetta12ORCID

Affiliation:

1. CNR Institute of Molecular Genetics “Luigi Luca Cavalli-Sforza”, Unit of Bologna, 40136 Bologna, Italy

2. IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy

3. Cellular Signalling Laboratory, Department of Biochemical and Neuromotor Sciences, Alma Mater Studiorum, University of Bologna, 40138 Bologna, Italy

4. Medicine and Rheumatology Unit, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy

5. Shoulder-Elbow Surgery Unit, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy

Abstract

In muscle cells subjected to mechanical stimulation, LINC complex and cytoskeletal proteins are basic to preserve cellular architecture and maintain nuclei orientation and positioning. In this context, the role of lamin A/C remains mostly elusive. This study demonstrates that in human myoblasts subjected to mechanical stretching, lamin A/C recruits desmin and plectin to the nuclear periphery, allowing a proper spatial orientation of the nuclei. Interestingly, in Emery-Dreifuss Muscular Dystrophy (EDMD2) myoblasts exposed to mechanical stretching, the recruitment of desmin and plectin to the nucleus and nuclear orientation were impaired, suggesting that a functional lamin A/C is crucial for the response to mechanical strain. While describing a new mechanism of action headed by lamin A/C, these findings show a structural alteration that could be involved in the onset of the muscle defects observed in muscular laminopathies.

Funder

AIDMEDproject

AIDMED-PROIETTI project

ECOSISTER Project

PRIN MUR

PRIN MUR ADIPOAGE

AIFA TREAT-LMNA

CNR project

Publisher

MDPI AG

Subject

General Medicine

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