Department of Physiology, Michigan State University, East Lansing, USA.
Genetics Program, Michigan State University, East Lansing, USA.
Commun Biol. 2020 Mar 16;3(1):127. doi: 10.1038/s42003-020-0833-4.
Fragile X syndrome (FXS) is a prevailing genetic disorder of intellectual disability and autism. There is no efficacious medication for FXS. Through in silico screening with a public database, computational analysis of transcriptome profile in FXS mouse neurons predicts therapeutic value of an FDA-approved drug trifluoperazine. Systemic administration of low-dose trifluoperazine at 0.05 mg/kg attenuates multiple FXS- and autism-related behavioral symptoms. Moreover, computational analysis of transcriptome alteration caused by trifluoperazine suggests a new mechanism of action against PI3K (Phosphatidylinositol-4,5-bisphosphate 3-kinase) activity. Consistently, trifluoperazine suppresses PI3K activity and its down-stream targets Akt (protein kinase B) and S6K1 (S6 kinase 1) in neurons. Further, trifluoperazine normalizes the aberrantly elevated activity of Akt and S6K1 and enhanced protein synthesis in FXS mouse. Together, our data demonstrate a promising value of transcriptome-based computation in identification of therapeutic strategy and repurposing drugs for neurological disorders, and suggest trifluoperazine as a potential treatment for FXS.
脆性 X 综合征(FXS)是一种普遍存在的智力障碍和自闭症遗传疾病。目前 FXS 没有有效的治疗药物。通过公共数据库的计算机筛选和 FXS 小鼠神经元转录组谱的计算分析,预测了一种已获 FDA 批准的药物三氟拉嗪的治疗价值。低剂量三氟拉嗪(0.05mg/kg)的全身给药可减轻多种 FXS 和自闭症相关的行为症状。此外,三氟拉嗪引起的转录组变化的计算分析表明了其针对 PI3K(磷脂酰肌醇-4,5-二磷酸 3-激酶)活性的新作用机制。一致地,三氟拉嗪抑制神经元中的 PI3K 活性及其下游靶标 Akt(蛋白激酶 B)和 S6K1(S6 激酶 1)。此外,三氟拉嗪可使 FXS 小鼠中异常升高的 Akt 和 S6K1 活性和增强的蛋白质合成恢复正常。总之,我们的数据证明了基于转录组计算在鉴定治疗策略和重新利用药物治疗神经疾病方面的有前途的价值,并表明三氟拉嗪可能是 FXS 的一种潜在治疗方法。