Division of Neuroscience and Experimental Psychology, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester M13 9PT, UK.
Institute of Neuroscience, University of Newcastle, Newcastle NE2 4HH, UK.
Dis Model Mech. 2022 Oct 1;15(10). doi: 10.1242/dmm.049703. Epub 2022 Oct 10.
Manipulating firing-rate neuronal homeostasis, which enables neurons to regulate their intrinsic excitability, offers an attractive opportunity to prevent seizures. However, to date, no drug-based interventions have been reported that manipulate this type of neuronal homeostatic mechanism. Here, we used a combination of Drosophila and mouse, and, in the latter, both a pentylenetetrazole (PTZ)-induced seizure model and an electrically induced seizure model for refractory seizures to evaluate the anticonvulsant efficacy of a novel class of anticonvulsant compounds, based on 4-tert-butyl-benzaldehyde (4-TBB). The mode of action included increased expression of the firing rate homeostatic regulator Pumilio (PUM). Knockdown of pum expression, in Drosophila, blocked anticonvulsive effects of 4-TBB, while analysis of validated PUM targets in mouse brain revealed significant reductions following exposure to this compound. A structure-activity study identified the active parts of the molecule and, further, showed that the pyrazole analogue demonstrates highest efficacy, being active against both PTZ-induced and electrically induced seizures. This study provides a proof of principle that anticonvulsant effects can be achieved through regulation of firing rate neuronal homeostasis and identifies a possible chemical compound for future development.
操纵神经元放电频率的内稳态,使神经元能够调节其固有兴奋性,这为预防癫痫发作提供了一个有吸引力的机会。然而,迄今为止,还没有报道任何基于药物的干预措施可以操纵这种类型的神经元内稳态机制。在这里,我们使用了果蝇和小鼠的组合,并且在后者中,我们使用了戊四氮(PTZ)诱导的癫痫发作模型和电诱导的难治性癫痫发作模型,来评估基于 4-叔丁基苯甲醛(4-TBB)的新型抗癫痫化合物的抗惊厥疗效。作用模式包括增加放电频率内稳态调节剂 Pumilio(PUM)的表达。在果蝇中敲低 pum 表达,阻断了 4-TBB 的抗惊厥作用,而在小鼠大脑中对经过验证的 PUM 靶点的分析表明,暴露于该化合物后,其表达显著减少。一项构效关系研究确定了该分子的活性部分,进一步表明,吡唑类似物具有最高的疗效,对 PTZ 诱导和电诱导的癫痫发作均有效。这项研究提供了一个原理证明,即通过调节神经元放电频率的内稳态可以实现抗惊厥作用,并确定了一种可能的化学化合物用于未来的开发。