Department of Biological Sciences, Middle East Technical University, Ankara, Türkiye.
Department of Pharmaceutical Chemistry, Faculty of Pharmacy, Gazi University, Ankara, Türkiye.
Drug Dev Res. 2024 Sep;85(6):e22248. doi: 10.1002/ddr.22248.
Botulinum neurotoxins (BoNT) inhibit neuroexocytosis, leading to the potentially lethal disease botulism. BoNT serotype A is responsible for most human botulism cases, and there are no approved therapeutics to treat already intoxicated patients. A growing body of research has demonstrated that BoNT/A can escape into the central nervous system, and therefore, identification of BoNT/A inhibitors that can penetrate BBB and neutralize the toxin within intoxicated neurons would be important. We previously identified an FDA-approved, orally bioavailable compound, KX2-391 (Tirbanibulin) that inhibits BoNT/A in motor neuron assays. Recently, a structural analog of KX2-391, KX2-361, has been shown to exhibit good oral bioavailability and cross BBB with high efficiency in mouse experiments. Therefore, in this work, we evaluated the inhibitory effects of KX2-361 against BoNT/A. Toward this goal, we first evaluated the compound for its effects on cell viability in PC12 cells, via MTT assay, and in mouse embryonic stem cell (mESC)-derived motor neurons, with imaging-based assays. Following, we tested KX2-361 in mESC-derived motor neurons intoxicated with BoNT/A holotoxin, and the compound exhibited activity against the toxin in both pre- and post-intoxication conditions. Excitingly, KX2-361 also inhibited BoNT/A enzymatic component (light chain; LC) in PC12 cells transfected with BoNT/A LC. Furthermore, our molecular docking analyses suggested that KX2-361 can directly bind to BoNT/A LC. Medicinal chemistry approaches to develop structural analogs of KX2-361 to increase its efficacy against BoNT/A may provide a critical lead compound with BBB penetration capacity for drug development efforts against BoNT/A intoxication.
肉毒神经毒素(BoNT)抑制神经递质的释放,从而导致潜在致命的疾病——肉毒中毒。BoNT 血清型 A 是引起大多数人类肉毒中毒的原因,目前尚无批准的治疗方法来治疗已中毒的患者。越来越多的研究表明,BoNT/A 可以逃逸到中枢神经系统,因此,鉴定能够穿透血脑屏障并中和中毒神经元内毒素的 BoNT/A 抑制剂将是很重要的。我们之前发现了一种已获得 FDA 批准的、可口服的化合物 KX2-391(替拉那布林),它可以在运动神经元检测中抑制 BoNT/A。最近,KX2-391 的结构类似物 KX2-361 已被证明在小鼠实验中具有良好的口服生物利用度和高效穿透血脑屏障的能力。因此,在这项工作中,我们评估了 KX2-361 对 BoNT/A 的抑制作用。为此,我们首先通过 MTT 测定法评估了该化合物对 PC12 细胞活力的影响,并通过基于成像的测定法评估了其对小鼠胚胎干细胞(mESC)衍生的运动神经元的影响。接着,我们在 BoNT/A 全毒素中毒的 mESC 衍生运动神经元中测试了 KX2-361,该化合物在中毒前和中毒后均表现出对毒素的活性。令人兴奋的是,KX2-361 还抑制了转染 BoNT/A LC 的 PC12 细胞中的 BoNT/A 酶组分(轻链;LC)。此外,我们的分子对接分析表明,KX2-361 可以直接与 BoNT/A LC 结合。开发 KX2-361 结构类似物的药物化学方法来提高其对 BoNT/A 的疗效,可能为开发针对 BoNT/A 中毒的穿透血脑屏障能力的关键先导化合物提供依据。