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Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients
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Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients

Published on: February 28, 2018

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Cyclophilin A in Arrhythmogenic Cardiomyopathy Cardiac Remodeling

Erica Rurali1, Chiara Assunta Pilato2, Gianluca Lorenzo Perrucci3

  • 1Unit of Vascular Biology and Regenerative Medicine, Centro Cardiologico Monzino IRCCS, 20138 Milano, Italy. erica.rurali@ccfm.it.

International Journal of Molecular Sciences
|May 18, 2019
PubMed

Insights

Cyclophilin A (CyPA) is elevated in arrhythmogenic cardiomyopathy (ACM) patients, correlating with fibro-fatty tissue. This protein is upregulated during cardiac mesenchymal stromal cell (C-MSC) adipogenesis, suggesting a role in ACM remodeling.

Area of Science:

  • Cardiovascular Biology
  • Genetic Disorders
  • Cellular Pathophysiology

Background:

  • Arrhythmogenic cardiomyopathy (ACM) involves fibro-fatty tissue replacement in the myocardium, leading to arrhythmias and sudden death.
  • Cyclophilin A (CyPA) is implicated in fibrosis, inflammation, and adipogenesis, processes relevant to ACM.
  • The role of CyPA in ACM cardiac remodeling remains unexplored.

Purpose of the Study:

  • To investigate Cyclophilin A (CyPA) expression in arrhythmogenic cardiomyopathy (ACM) cardiac remodeling.
  • To assess CyPA modulation in ACM-derived cardiac mesenchymal stromal cells (C-MSCs) during adipogenesis.

Main Methods:

  • Immunohistochemistry was used to evaluate RV tissue from ACM patients and healthy controls (HC).
  • ACM- and HC-derived C-MSCs were utilized to study CyPA expression and secretion during adipogenic differentiation.

Main Results:

  • CyPA expression was significantly higher in the right ventricle (RV) of ACM patients compared to HC.
  • Elevated CyPA levels in ACM correlated with the extent of adipose tissue replacement.
  • CyPA was upregulated during early adipogenic differentiation of C-MSCs and secreted at higher levels by ACM-derived C-MSCs.

Conclusions:

  • This study reveals increased CyPA expression in ACM cardiac tissue and its upregulation during adipogenesis.
  • Findings suggest CyPA plays a role in ACM-associated cardiac remodeling.
  • Provides a basis for future C-MSC-based research into ACM mechanisms and therapies.

Keywords:
adipogenesisarrhythmogenic cardiomyopathycardiac mesenchymal stromal cellscyclophilin Afibrosis

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