整合组学,探索唐氏综合征神经退行性变的新疾病途径-专注于综合应激反应

Beatriz Barros-Santos , Carlos Campos-Marques , Andreia Filipa Salvador , Joana Margarida Silva
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引用次数: 0

摘要

唐氏综合症的特征是21号染色体三体,导致广泛的分子和神经改变,包括早发性阿尔茨海默病。新出现的证据强调了RNA代谢、RNA结合蛋白和应激颗粒在这些过程中的作用。综合应激反应是翻译和蛋白质稳态的关键调节因子,由于参与蛋白质降解和RNA转录平衡的基因过度表达,在DS中可能特别受到破坏。然而,其对退行性椎体滑移神经退行性变的影响仍然知之甚少。该项目旨在整合来自唐氏综合征人类和动物模型的转录组学和蛋白质组学数据,以剖析综合应激反应、rna结合蛋白和应激颗粒动力学之间的相互作用。通过确定RNA稳态和蛋白质合成中的关键分子破坏,我们的目标是研究可以在物种之间保守的新型疾病驱动机制。这些见解可能有助于将ISR作为潜在的治疗靶点,促进我们对DS相关神经退行性通路的理解,这些通路可能是DS中观察到的与年龄相关的神经退行性变的背后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Integration of omics to explore novel disease pathways in Down Syndrome neurodegeneration – Focusing on integrated stress response
Down Syndrome is characterized by the trisomy of chromosome 21, leading to widespread molecular and neurological alterations, including early-onset Alzheimer's disease. Emerging evidence highlights the role of RNA metabolism, RNA-binding proteins, and stress granules in these processes. The integrated stress response, a key regulator of translation and protein homeostasis, may be particularly disrupted in DS due to the overexpression of genes involved in the balance between protein degradation and RNA transcription. However, its impact on neurodegeneration in DS remains poorly understood. This project aims to integrate transcriptomic and proteomic data from human and animal models with Down Syndrome to dissect the interplay between integrated stress response, RNA-binding proteins, and stress granule dynamics. By identifying key molecular disruptions in RNA homeostasis and protein synthesis, we aim to investigate novel disease-driving mechanisms that can be conserved among species. These insights will likely help to establish ISR as a potential therapeutic target, advancing our understanding of DS-related neurodegenerative pathways that could be behind the age-related neurodegeneration observed in DS.
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