Castonguay Charles-Etienne, Liao Calwing, Khayachi Anouar, Liu Yumin, Medeiros Miranda, Houle Gabrielle, Ross Jay P, Dion Patrick A, Rouleau Guy A
Department of Human Genetics, McGill University, Montreal, QC, Canada.
Montreal Neurological Institute, Department of Neurology and Neurosurgery, McGill University, Montreal, QC, Canada.
NPJ Genom Med. 2022 Aug 4;7(1):46. doi: 10.1038/s41525-022-00318-9.
Essential tremor (ET) is one of the most common movement disorders, affecting nearly 5% of individuals over 65 years old. Despite this, few genetic risk loci for ET have been identified. Recent advances in pharmacogenomics have previously been useful to identify disease related molecular targets. Notably, gene expression has proven to be quite successful for the inference of drug response in cell models. We sought to leverage this approach in the context of ET where many patients are responsive to two drugs: propranolol and primidone. In this study, cerebellar DAOY and neural progenitor cells were treated for 5 days with clinical concentrations of propranolol and primidone, after which RNA-sequencing was used to identify convergent differentially expressed genes across treatments. Propranolol was found to affect the expression of genes previously associated with ET and other movement disorders such as TRAPPC11. Pathway enrichment analysis of these convergent drug-targeted genes identified multiple terms related to calcium signaling, endosomal sorting, axon guidance, and neuronal morphology. Furthermore, genes targeted by ET drugs were enriched within cell types having high expression of ET-related genes in both cortical and cerebellar tissues. Altogether, our results highlight potential cellular and molecular mechanisms associated with tremor reduction and identify relevant genetic biomarkers for drug-responsiveness in ET.
特发性震颤(ET)是最常见的运动障碍之一,影响着近5%的65岁以上人群。尽管如此,已确定的ET遗传风险位点却很少。药物基因组学的最新进展此前已有助于识别与疾病相关的分子靶点。值得注意的是,基因表达已被证明在细胞模型中推断药物反应方面非常成功。我们试图在ET的背景下利用这种方法,因为许多患者对两种药物有反应:普萘洛尔和扑米酮。在这项研究中,小脑DAOY细胞和神经祖细胞用临床浓度的普萘洛尔和扑米酮处理5天,之后使用RNA测序来识别不同处理之间趋同的差异表达基因。发现普萘洛尔会影响先前与ET和其他运动障碍相关的基因表达,如TRAPPC11。这些趋同的药物靶向基因的通路富集分析确定了多个与钙信号传导、内体分选、轴突导向和神经元形态相关的术语。此外,ET药物靶向的基因在皮质和小脑组织中高表达ET相关基因的细胞类型中富集。总之,我们的结果突出了与震颤减轻相关的潜在细胞和分子机制,并确定了ET中药物反应性的相关遗传生物标志物。