Creighton University School of Medicine, Omaha, NE 68178, USA.
Institute of Physiology, University of Zürich, Zürich CH-8057, Switzerland.
Sci Transl Med. 2024 Aug 7;16(759):eadn2140. doi: 10.1126/scitranslmed.adn2140.
Hearing loss is a major health concern in our society, affecting more than 400 million people worldwide. Among the causes, aminoglycoside therapy can result in permanent hearing loss in 40% to 60% of patients receiving treatment, and despite these high numbers, no drug for preventing or treating this type of hearing loss has yet been approved by the US Food and Drug Administration. We have previously conducted high-throughput screenings of bioactive compounds, using zebrafish as our discovery platform, and identified piplartine as a potential therapeutic molecule. In the present study, we expanded this work and characterized piplartine's physicochemical and therapeutic properties. We showed that piplartine had a wide therapeutic window and neither induced nephrotoxicity in vivo in zebrafish nor interfered with aminoglycoside antibacterial activity. In addition, a fluorescence-based assay demonstrated that piplartine did not inhibit cytochrome C activity in microsomes. Coadministration of piplartine protected from kanamycin-induced hair cell loss in zebrafish and protected hearing function, outer hair cells, and presynaptic ribbons in a mouse model of kanamycin ototoxicity. Last, we investigated piplartine's mechanism of action by phospho-omics, immunoblotting, immunohistochemistry, and molecular dynamics experiments. We found an up-regulation of AKT1 signaling in the cochleas of mice cotreated with piplartine. Piplartine treatment normalized kanamycin-induced up-regulation of TRPV1 expression and modulated the gating properties of this receptor. Because aminoglycoside entrance to the inner ear is, in part, mediated by TRPV1, these results suggested that by regulating TRPV1 expression, piplartine blocked aminoglycoside's entrance, thereby preventing the long-term deleterious effects of aminoglycoside accumulation in the inner ear compartment.
听力损失是我们社会中的一个主要健康问题,影响着全球超过 4 亿人。在这些原因中,氨基糖苷类药物治疗会导致接受治疗的患者中有 40%至 60%出现永久性听力损失,尽管这个数字很高,但美国食品和药物管理局尚未批准任何用于预防或治疗这种类型听力损失的药物。我们之前曾使用斑马鱼作为发现平台,对生物活性化合物进行高通量筛选,鉴定出胡椒碱作为一种潜在的治疗分子。在本研究中,我们扩展了这项工作,对胡椒碱的理化性质和治疗特性进行了表征。我们表明,胡椒碱具有较宽的治疗窗口,既不会在体内诱导斑马鱼的肾毒性,也不会干扰氨基糖苷类药物的抗菌活性。此外,荧光测定表明胡椒碱不会抑制微粒体中的细胞色素 C 活性。胡椒碱与卡那霉素共同给药可保护斑马鱼免受卡那霉素诱导的毛细胞损失,并可保护听力功能、外毛细胞和卡那霉素耳毒性小鼠模型中的突触前带。最后,我们通过磷酸化组学、免疫印迹、免疫组织化学和分子动力学实验研究了胡椒碱的作用机制。我们发现,用胡椒碱共同处理的小鼠耳蜗中 AKT1 信号上调。胡椒碱处理使 TRPV1 表达的卡那霉素诱导上调正常化,并调节了该受体的门控特性。因为氨基糖苷类药物进入内耳的部分是由 TRPV1 介导的,这些结果表明,通过调节 TRPV1 的表达,胡椒碱阻断了氨基糖苷类药物的进入,从而防止了氨基糖苷类药物在内耳部分的积累产生长期的有害影响。