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RNA Deregulation in Amyotrophic Lateral Sclerosis: The Noncoding Perspective

Journal

Publisher

MDPI
DOI: 10.3390/ijms221910285

Keywords

ALS; motoneurons; neurodegeneration; RNA metabolism; noncoding RNAs; microRNAs; long noncoding RNAs; circular RNAs

Funding

  1. PRIN 2017 [2017P352Z4]

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RNA metabolism is crucial to cellular physiopathology, with ncRNAs playing a key role in maintaining neural system homeostasis and potentially contributing to neurodegenerative diseases like ALS. Research explores the link between ncRNA dysregulation and ALS, presenting the possibility of using them as diagnostic biomarkers and therapeutic targets.
RNA metabolism is central to cellular physiopathology. Almost all the molecular pathways underpinning biological processes are affected by the events governing the RNA life cycle, ranging from transcription to degradation. The deregulation of these processes contributes to the onset and progression of human diseases. In recent decades, considerable efforts have been devoted to the characterization of noncoding RNAs (ncRNAs) and to the study of their role in the homeostasis of the nervous system (NS), where they are highly enriched. Acting as major regulators of gene expression, ncRNAs orchestrate all the steps of the differentiation programs, participate in the mechanisms underlying neural functions, and are crucially implicated in the development of neuronal pathologies, among which are neurodegenerative diseases. This review aims to explore the link between ncRNA dysregulation and amyotrophic lateral sclerosis (ALS), the most frequent motoneuron (MN) disorder in adults. Notably, defective RNA metabolism is known to be largely associated with this pathology, which is often regarded as an RNA disease. We also discuss the potential role that these transcripts may play as diagnostic biomarkers and therapeutic targets.

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