Dataset related to the article "Cyclophilin A in Arrhythmogenic Cardiomyopathy Cardiac Remodeling"

This record contains raw data related to the article "Cyclophilin A in Arrhythmogenic Cardiomyopathy Cardiac Remodeling"

Abstract: 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.

本数据集包含与论文《致心律失常性心肌病心肌重构中的亲环蛋白A》相关的原始数据。

摘要：致心律失常性心肌病（Arrhythmogenic cardiomyopathy, ACM）是一种遗传性疾病，其特征为功能性心肌被无收缩功能的纤维脂肪组织进行性替代，进而引发室性心律失常与心源性猝死。亲环蛋白A（Cyclophilin A, CyPA）是一种普遍存在的蛋白质，参与多种病理过程，而这些病理过程同样是致心律失常性心肌病的典型特征，即纤维化、炎症与脂肪生成。然而，亲环蛋白A在致心律失常性心肌病心肌重构中的作用尚未得到研究。因此，本研究首先通过免疫组织化学法，检测了ACM患者与健康对照（healthy controls, HC）的右心室（right ventricle, RV）组织样本中的亲环蛋白A表达水平。随后，研究利用ACM患者及健康对照来源的心脏间充质基质细胞（cardiac mesenchymal stromal cells, C-MSC），探究脂肪生成分化过程中亲环蛋白A的调控变化。值得注意的是，与健康对照相比，ACM患者的右心室组织切片中亲环蛋白A的表达水平更高，且与脂肪组织替代程度呈正相关。此外，在心脏间充质基质细胞的脂肪生成分化早期，亲环蛋白A的表达会上调，且ACM来源的心脏间充质基质细胞中，亲环蛋白A的分泌水平随时间推移进一步升高。本研究为致心律失常性心肌病重构过程中亲环蛋白A的表达情况提供了全新的离体与体外研究数据，为后续基于心脏间充质基质细胞的机制研究与治疗探索奠定了基础。