Schmidt Elena N C, Evert Bernd O, Pregler Barbara E F, Melhem Ahmad, Hsieh Meng-Chun, Raspe Markus, Strobel Hannah, Roos Julian, Pietsch Torsten, Schuss Patrick, Fischer-Posovszky Pamela, Westhoff Mike-Andrew, Hölzel Michael, Herrlinger Ulrich, Vatter Hartmut, Waha Andreas, Schneider Matthias, Potthoff Anna-Laura
Department of Neurosurgery, University Hospital Bonn, Germany.
Brain Tumor Translational Research Group, University Hospital Bonn, Germany.
Mol Oncol. 2025 Mar;19(3):878-898. doi: 10.1002/1878-0261.13786. Epub 2024 Dec 16.
Glioblastoma cells rely on connexin 43 (Cx43)-based gap junctions (GJs) for intercellular communication, enabling them to integrate into a widely branched malignant network. Although there are promising prospects for new targeted therapies, the lack of clinically feasible GJ inhibitors has impeded their adoption in clinical practice. In the present study, we investigated tonabersat (TO), a blood-brain-barrier-penetrating drug with GJ-inhibitory properties, in regard to its potential to disassemble intercellular connectivity in glioblastoma networks. Fluorescence-guided measurements of calcein cell-to-cell transfer were used to study functional intercellular connectivity. Specific DNA fragmentation rates of propidium iodide-stained nuclei were measured as a surrogate readout for cell death using flow cytometry. CRISPR/Cas9-mediated gene editing of Cx43 served as a validation tool of cellular effects related to Cx43 GJ inhibition. 3' mRNA sequencing was performed for molecular downstream analysis. We found that TO reduced intercellular GJ-mediated cytosolic traffic and yielded a significant reduction of tumor microtube (TM) length. TO-mediated inhibition of cellular tumor networks was accompanied by a synergistic effect for temozolomide-induced cell death. CRISPR/Cas9 Cx43-knockout revealed similar results, indicating that TO-mediated inhibitory effects rely on the inhibition of Cx43-based GJs. Gene set enrichment analyses found that GJ-mediated synergistic cytotoxic effects were linked to a significant upregulation of cell death signaling pathways. In conclusion, TO disrupts TM-based network connectivity via GJ inhibition and renders glioblastoma cells more susceptible to cytotoxic therapy. Given its previous use in clinical trials for migraine therapy, TO might harbor the potential of bridging the idea of a GJ-targeted therapeutic approach from bench to bedside.
胶质母细胞瘤细胞依靠基于连接蛋白43(Cx43)的间隙连接(GJs)进行细胞间通讯,使其能够整合到广泛分支的恶性网络中。尽管新的靶向治疗前景广阔,但缺乏临床可行的GJ抑制剂阻碍了它们在临床实践中的应用。在本研究中,我们研究了托纳贝萨特(TO),一种具有GJ抑制特性的血脑屏障穿透药物,探讨其拆散胶质母细胞瘤网络中细胞间连接的潜力。使用钙黄绿素细胞间转移的荧光引导测量来研究功能性细胞间连接。使用流式细胞术测量碘化丙啶染色细胞核的特定DNA片段化率,作为细胞死亡的替代读数。Cx43的CRISPR/Cas9介导的基因编辑用作与Cx43 GJ抑制相关的细胞效应的验证工具。进行3' mRNA测序以进行分子下游分析。我们发现TO减少了细胞间GJ介导的胞质运输,并使肿瘤微管(TM)长度显著缩短。TO介导的细胞肿瘤网络抑制伴随着替莫唑胺诱导的细胞死亡的协同效应。CRISPR/Cas9 Cx43基因敲除显示了相似的结果,表明TO介导的抑制作用依赖于对基于Cx43的GJs的抑制。基因集富集分析发现,GJ介导的协同细胞毒性作用与细胞死亡信号通路的显著上调有关。总之,TO通过抑制GJ破坏基于TM的网络连接,并使胶质母细胞瘤细胞对细胞毒性治疗更敏感。鉴于其先前在偏头痛治疗临床试验中的应用,TO可能具有将GJ靶向治疗方法从实验室转化到临床应用的潜力。