Cyclophilin a in arrhythmogenic cardiomyopathy cardiac remodeling

Erica Rurali, Chiara Assunta Pilato, Gianluca Lorenzo Perrucci, Alessandro Scopece, Ilaria Stadiotti, Donato Moschetta, Michela Casella, Elisa Cogliati, Elena Sommariva, Giulio Pompilio, Patrizia Nigro

Research output: Contribution to journalArticlepeer-review


Arrhythmogenic cardiomyopathy (ACM) is a genetic disorder characterized by the progressive substitution of functional myocardium with noncontractile fibro-fatty tissue contributing to ventricular arrhythmias and sudden cardiac death. Cyclophilin A (CyPA) is a ubiquitous protein involved in several pathological mechanisms, which also characterize ACM (i.e., fibrosis, inflammation, and adipogenesis). Nevertheless, the involvement of CyPA in ACM cardiac remodeling has not been investigated yet. Thus, we first evaluated CyPA expression levels in the right ventricle (RV) tissue specimens obtained from ACM patients and healthy controls (HC) by immunohistochemistry. Then, we took advantage of ACM-and HC-derived cardiac mesenchymal stromal cells (C-MSC) to assess CyPA modulation during adipogenic differentiation. Interestingly, CyPA was more expressed in the RV sections obtained from ACM vs. HC subjects and positively correlated with the adipose replacement extent. Moreover, CyPA was upregulated at early stages of C-MSC adipogenic differentiation and was secreted at higher level over time in ACM-derived C-MSC. Our study provides novel ex vivo and in vitro information on CyPA expression in ACM remodeling paving the way for future C-MSC-based mechanistic and therapeutic investigations.

Original languageEnglish
Article number2403
JournalInternational Journal of Molecular Sciences
Issue number10
Publication statusPublished - May 2 2019


  • Adipogenesis
  • Arrhythmogenic cardiomyopathy
  • Cardiac mesenchymal stromal cells
  • Cyclophilin A
  • Fibrosis

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry


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