Garg Prekshi, Jamal Farrukh, Srivastava Prachi
Amity Institute of Biotechnology, Amity University Uttar Pradesh, Lucknow Campus, 226028, India.
Department of Biochemistry, Dr. Rammanohar Lohia Avadh University, Ayodhya, 224001, UP, India.
Heliyon. 2024 May 4;10(9):e30755. doi: 10.1016/j.heliyon.2024.e30755. eCollection 2024 May 15.
Intellectual disability (ID) is a progressive disorder that affects around 1-3% of the world's population. The heterogeneity of intellectual disability makes it difficult to diagnose as a complete disease. Genetic factors and major mutations play a noticeable role in the development and progression of ID. There is a high need to explore novel variants that may lead to new insights into the progressive aspects of ID. In the current course of study, 31 samples of ID from different studies available on GEO (GSE77742, GSE74263, GSE90682, GSE98476, GSE108887, GSE145710, and PRJEB21964) datasets were taken for the study. These datasets were analyzed for differential gene expression and single nucleotide polymorphism (SNPs). The SNPs of high impact were compared with the differentially expressed genes. Comparison leads to the identification of the priority gene ie NPR3 gene. The identified priority gene further was evaluated for the effect of the mutation using a Mutation Taster. Structure comparison analysis of the wild and mutated proteins of the NPR3 gene was further carried out by UCSF Chimera. Structural analysis reveals the anomalies in protein expression affecting the regulations of the NPR3 gene. These findings identified a novel nonsense mutation (E222*) in the downregulated NPR3 gene that leads to anomalies in the regulation of its protein expression. This missense mutation reveals a major role in causing ID. Our study concludes that the decrease in the expression of the NPR3 gene causes delayed sensory, motor, and physiological functions of the human brain leading to neurodevelopmental delay that causes ID.
智力残疾(ID)是一种渐进性疾病,影响着全球约1%-3%的人口。智力残疾的异质性使其难以被诊断为一种完整的疾病。遗传因素和主要突变在智力残疾的发生和发展中起着显著作用。迫切需要探索可能为智力残疾的渐进性方面带来新见解的新变异。在当前的研究过程中,从GEO(GSE77742、GSE74263、GSE90682、GSE98476、GSE108887、GSE145710和PRJEB21964)数据集中选取了来自不同研究的31个智力残疾样本进行研究。对这些数据集进行了差异基因表达和单核苷酸多态性(SNP)分析。将高影响的SNP与差异表达基因进行比较。比较结果确定了优先基因,即 NPR3基因。使用突变预测工具对鉴定出的优先基因的突变效应进行了进一步评估。通过UCSF Chimera对NPR3基因的野生型和突变型蛋白质进行了结构比较分析。结构分析揭示了影响NPR3基因调控的蛋白质表达异常。这些发现确定了下调的NPR3基因中的一种新的无义突变(E222*),该突变导致其蛋白质表达调控异常。这种错义突变在导致智力残疾方面起着重要作用。我们的研究得出结论,NPR3基因表达的降低导致人类大脑的感觉、运动和生理功能延迟,从而导致神经发育迟缓,进而引发智力残疾。