Perdana University Graduate School of Medicine, Perdana University, Kuala Lumpur, Malaysia.
Department of Research and Training, MAHSA Specialist Hospital, Selangor, Malaysia.
J Neuropathol Exp Neurol. 2024 Oct 1;83(10):822-832. doi: 10.1093/jnen/nlae065.
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder due to deletion or mutation of survival motor neuron 1 (SMN1) gene. Although survival motor neuron 2 (SMN2) gene is still present in SMA patients, the production of full-length survival motor neuron (SMN) protein is insufficient owing to missing or mutated SMN1. No current disease-modifying therapies can cure SMA. The aim of this study was to explore microRNA (miRNA)-based therapies that may serve as a potential target for therapeutic intervention in delaying SMA progression or as treatment. The study screened for potentially dysregulated miRNAs in SMA fibroblast-derived iPSCs using miRNA microarray. Results from the miRNA microarray were validated using quantitative reverse transcription polymerase chain reaction. Bioinformatics analysis using various databases was performed to predict the potential putative gene targeted by hsa-miR-663a. The findings showed differential expression of hsa-miR-663a in SMA patients in relation to a healthy control. Bioinformatics analysis identified GNG7, IGF2, and TNN genes that were targeted by hsa-miR-663a to be involved in the PI3K-AKT pathway, which may be associated with disease progression in SMA. Thus, this study suggests the potential role of hsa-miR-663a as therapeutic target for the treatment of SMA patients in the near future.
脊髓性肌萎缩症(SMA)是一种常染色体隐性神经肌肉疾病,由于生存运动神经元 1(SMN1)基因缺失或突变所致。尽管 SMA 患者仍存在生存运动神经元 2(SMN2)基因,但由于 SMN1 缺失或突变,全长生存运动神经元(SMN)蛋白的产生不足。目前尚无针对 SMA 的疾病修正疗法可以治愈该疾病。本研究旨在探索基于 microRNA(miRNA)的治疗方法,这些方法可能成为治疗 SMA 进展或作为治疗方法的潜在治疗靶点。本研究使用 miRNA 微阵列筛选 SMA 成纤维细胞衍生的诱导多能干细胞中可能失调的 miRNA。使用定量逆转录聚合酶链反应验证 miRNA 微阵列的结果。使用各种数据库进行生物信息学分析,以预测 hsa-miR-663a 潜在的靶基因。研究结果显示,与健康对照组相比,SMA 患者中 hsa-miR-663a 的表达存在差异。生物信息学分析鉴定了 GNG7、IGF2 和 TNN 基因是 hsa-miR-663a 的靶基因,这些基因参与了 PI3K-AKT 通路,这可能与 SMA 疾病进展有关。因此,本研究提示 hsa-miR-663a 可能作为 SMA 患者治疗靶点的潜在作用,有望在不久的将来用于治疗 SMA 患者。