Dept. of Cardiology and Pneumology, CharitéCentrum11 (Cardiovascular Medicine), CBF, Germany.
Institute for Biometry and Clinical Epidemiology, CBF, Germany.
J Mol Cell Cardiol. 2014 Jan;66:141-56. doi: 10.1016/j.yjmcc.2013.11.004. Epub 2013 Nov 12.
Therapeutic targets of broad relevance are likely located in pathogenic pathways common to disorders of various etiologies. Screening for targets of this type revealed CCN genes to be consistently upregulated in multiple cardiomyopathies. We developed RNA interference (RNAi) to silence CCN2 and found this single-target approach to block multiple proinflammatory and profibrotic pathways in activated primary cardiac fibroblasts (PCFBs). The RNAi-strategy was developed in murine PCFBs and then investigated in "individual" human PCFBs grown from human endomyocardial biopsies (EMBs). Screening of short hairpin RNA (shRNA) sequences for high silencing efficacy and specificity yielded RNAi adenovectors silencing CCN2 in murine or human PCFBs, respectively. Comparison of RNAi with CCN2-modulating microRNA (miR) vectors expressing miR-30c or miR-133b showed higher efficacy of RNAi. In murine PCFBs, CCN2 silencing resulted in strongly reduced expression of stretch-induced chemokines (Ccl2, Ccl7, Ccl8), matrix metalloproteinases (MMP2, MMP9), extracellular matrix (Col3a1), and a cell-to-cell contact protein (Cx43), suggesting multiple signal pathways to be linked to CCN2. Immune cell chemotaxis towards CCN2-depleted PCFBs was significantly reduced. We demonstrate here that this RNAi strategy is technically applicable to "individual" human PCFBs, too, but that these display individually strikingly different responses to CCN2 depletion. Either genomically encoded factors or stable epigenetic modification may explain different responses between individual PCFBs. The new RNAi approach addresses a key regulator protein induced in cardiomyopathies. Investigation of this and other molecular therapies in individual human PCBFs may help to dissect differential pathogenic processes between otherwise similar disease entities and individuals.
广泛相关的治疗靶点可能位于各种病因的疾病共有的致病途径中。针对此类靶点的筛选表明,CCN 基因在多种心肌病中持续上调。我们开发了 RNA 干扰 (RNAi) 来沉默 CCN2,并发现这种单一靶点方法可阻断激活的原代心肌成纤维细胞 (PCFB) 中的多种促炎和促纤维化途径。该 RNAi 策略在鼠源 PCFB 中开发,然后在从人类心内膜活检 (EMB) 中培养的“个体”人类 PCFB 中进行研究。筛选短发夹 RNA (shRNA) 序列以实现高沉默效率和特异性,得到了分别在鼠源或人源 PCFB 中沉默 CCN2 的 RNAi 腺病毒载体。比较 RNAi 与表达 miR-30c 或 miR-133b 的 CCN2 调节 microRNA (miR) 载体,结果显示 RNAi 的效果更高。在鼠源 PCFB 中,CCN2 沉默导致拉伸诱导的趋化因子 (Ccl2、Ccl7、Ccl8)、基质金属蛋白酶 (MMP2、MMP9)、细胞外基质 (Col3a1) 和细胞间接触蛋白 (Cx43) 的表达显著降低,表明多个信号通路与 CCN2 相关。CCN2 耗尽的 PCFB 对免疫细胞的趋化作用明显降低。我们在此证明,该 RNAi 策略也可在“个体”人源 PCFB 中应用,但这些细胞对 CCN2 耗竭的反应个体差异明显。可能是基因组编码的因素或稳定的表观遗传修饰解释了个体 PCFB 之间的不同反应。新的 RNAi 方法针对心肌病中诱导的关键调节蛋白。在个体人类 PCBF 中研究这种和其他分子疗法可能有助于剖析不同疾病实体和个体之间的差异致病过程。