Project

REMIND

Role of the extracellular matrix in the regulation of cardiac sympathetic innervation in Duchenne muscular dystrophy

ITB Principal Investigator

Name

Role of the extracellular matrix in the regulation of cardiac sympathetic innervation in Duchenne muscular dystrophy

Acronym

REMIND

Location

Segrate

Start Date

2023

End Date

2026

Funder

National Research Council (CNR)

Partners

- "Sapienza" University of Rome, Rome, Italy (Prof. R. Rizzi) - Institute of Physical Chemistry - Polish Academy of Sciences, Warsaw, Poland (Dr M. Costantini) - Ludwig Maximilian University of Munich, Munich, Germany (Prof. E. Wolf)

The project investigates the mechanisms linking Duchenne muscular dystrophy (DMD) to the progressive decline of cardiac function, with a particular focus on the role of the extracellular matrix (ECM). In DMD, the absence of dystrophin not only affects muscle tissue but also disrupts the balance among complex systems involving skeletal muscle, the heart, and the nervous system. In this context, the accumulation of specific ECM components appears to promote dysfunction of the sympathetic neurons that regulate the heart, contributing to the loss of proper muscle tone and the onset of arrhythmias. Based on the hypothesis that ECM composition may be capable of inhibiting sympathetic cardiac innervation, the project seeks to clarify the pathological role of these processes and to develop innovative strategies to restore cardiac innervation. To achieve this, the study will employ advanced 3D cultures, enabling the creation of highly representative experimental models and a more precise characterization of the contribution of the ECM to cardiac deterioration in DMD.
  • Milan M, et al. Macrophages producing chondroitin sulfate proteoglycan-4 induce neuro-cardiac junction impairment in Duchenne muscular dystrophy. J Pathol (2025) 265(1):1-13. doi: 10.1002/path. 6362.