Department of Pharmaceutical Sciences, UNT System College of Pharmacy, University of North Texas Health Science Center, Fort Worth, TX 76107, USA; School of Biomedical Sciences, University of North Texas Health Science Center, Fort Worth, TX 76107, USA.
Department of Pharmacology, Vanderbilt University, Nashville, TN 37232, USA.
Bioorg Med Chem. 2023 Nov 15;95:117487. doi: 10.1016/j.bmc.2023.117487. Epub 2023 Sep 30.
Malignant migrating partial seizure of infancy (MMPSI) is a devastating and pharmacoresistant form of infantile epilepsy. MMPSI has been linked to multiple gain-of-function (GOF) mutations in the KCNT1 gene, which encodes for a potassium channel often referred to as SLACK. SLACK channels are sodium-activated potassium channels distributed throughout the central nervous system (CNS) and the periphery. The investigation described here aims to discover SLACK channel inhibitor tool compounds and profile their pharmacokinetic and pharmacodynamic properties. A SLACK channel inhibitor VU0531245 (VU245) was identified via a high-throughput screen (HTS) campaign. Structure-activity relationship (SAR) studies were conducted in five distinct regions of the hit VU245. VU245 analogs were evaluated for their ability to affect SLACK channel activity using a thallium flux assay in HEK-293 cells stably expressing wild-type (WT) human SLACK. Selected analogs were tested for metabolic stability in mouse liver microsomes and plasma-protein binding in mouse plasma. The same set of analogs was tested via thallium flux for activity versus human A934T SLACK and other structurally related potassium channels, including SLICK and Maxi-K. In addition, potencies for selected VU245 analogs were obtained using whole-cell electrophysiology (EP) assays in CHO cells stably expressing WT human SLACK through an automated patch clamp system. Results revealed that this scaffold tolerates structural changes in some regions, with some analogs demonstrating improved SLACK inhibitory activity, good selectivity against the other channels tested, and modest improvements in metabolic clearance. Analog VU0935685 represents a new, structurally distinct small-molecule inhibitor of SLACK channels that can serve as an in vitro tool for studying this target.
婴儿进行性部分性癫痫伴游走性阵挛发作(MMPSI)是一种毁灭性的、药物难治性婴儿癫痫形式。MMPSI 与 KCNT1 基因的多种获得性功能(GOF)突变有关,该基因编码一种钾通道,通常称为 SLACK。SLACK 通道是分布在中枢神经系统(CNS)和外周的钠激活钾通道。这里描述的研究旨在发现 SLACK 通道抑制剂工具化合物,并分析其药代动力学和药效动力学特性。通过高通量筛选(HTS)活动,鉴定出 SLACK 通道抑制剂 VU0531245(VU245)。在命中 VU245 的五个不同区域进行了结构活性关系(SAR)研究。使用在稳定表达野生型(WT)人 SLACK 的 HEK-293 细胞中进行的铊通量测定,评估 VU245 类似物对 SLACK 通道活性的影响。选择的类似物在小鼠肝微粒体中进行代谢稳定性测试,并在小鼠血浆中进行血浆蛋白结合测试。对同一组类似物进行铊通量测试,以测试其对人 A934T SLACK 和其他结构相关钾通道(包括 SLICK 和 Maxi-K)的活性。此外,通过在稳定表达 WT 人 SLACK 的 CHO 细胞中进行全细胞电生理学(EP)测定,获得了选定的 VU245 类似物的部分活性。结果表明,该支架可以容忍某些区域的结构变化,一些类似物显示出更好的 SLACK 抑制活性,对测试的其他通道具有良好的选择性,以及代谢清除率的适度提高。类似物 VU0935685 代表了一种新的、结构独特的 SLACK 通道小分子抑制剂,可作为研究该靶标的体外工具。