Advanced Clinical Biosystems Research Institute, Smidt Heart Institute.
Precision Biomarker Laboratories, Cedars-Sinai Medical Center, Los Angeles, California.
J Histochem Cytochem. 2024 Jun;72(6):387-397. doi: 10.1369/00221554241252862. Epub 2024 May 16.
During investigating the role of peptidylarginine deiminase (PAD) enzymes in dilated cardiomyopathy (DCM), we observed unique spheroid formation in DCM-myofibroblasts that distinguished them from normal cardiac myofibroblasts. The present study aimed to assess the presence of PADs, the extracellular matrix (ECM), and citrullination in DCM spheroids using immunofluorescence staining and imaging techniques. The results revealed that spheroids derived from DCM-myofibroblasts displayed a more distinctive, tightly packed structure compared with those derived from human cardiac fibroblasts. DCM spheroids showed abundant protein expression of the PAD 2, 3, and 4 enzymes. Notably, increased Ki67 protein expression was associated with increased proliferation in DCM spheroids. Cytoskeletal proteins such as Col-1A, vimentin, α-SMA, and F-actin were highly abundant in DCM spheroids. Furthermore, DCM spheroids contained citrullinated cytoskeletal proteins, mainly citrullinated vimentin and citrullinated fibronectin. These observations supported the occurrence of PAD-mediated citrullination of ECM proteins in DCM spheroids. Collectively, these findings describe the distinctive features of DCM spheroids, representing the cellular characteristics of DCM myofibroblasts. Therefore, DCM spheroids can serve as an in vitro model for further investigations of disease morphology and therapeutic efficacy.
在研究肽基精氨酸脱亚氨酶(PAD)酶在扩张型心肌病(DCM)中的作用时,我们观察到 DCM 肌成纤维细胞中存在独特的球体形成,这将它们与正常的心肌成纤维细胞区分开来。本研究旨在使用免疫荧光染色和成像技术评估 DCM 球体中的 PAD、细胞外基质(ECM)和瓜氨酸化的存在情况。结果表明,与来源于人心肌成纤维细胞的球体相比,来源于 DCM 肌成纤维细胞的球体显示出更独特、更紧密的结构。DCM 球体显示出 PAD2、3 和 4 酶的丰富蛋白表达。值得注意的是,DCM 球体中 Ki67 蛋白表达的增加与增殖增加相关。细胞骨架蛋白,如 Col-1A、波形蛋白、α-SMA 和 F-肌动蛋白在 DCM 球体中高度丰富。此外,DCM 球体含有瓜氨酸化的细胞骨架蛋白,主要是瓜氨酸化的波形蛋白和瓜氨酸化的纤维连接蛋白。这些观察结果支持 DCM 球体中 PAD 介导的 ECM 蛋白瓜氨酸化的发生。总之,这些发现描述了 DCM 球体的独特特征,代表了 DCM 肌成纤维细胞的细胞特征。因此,DCM 球体可以作为进一步研究疾病形态和治疗效果的体外模型。