IMR Press / FBE / Volume 10 / Issue 1 / DOI: 10.2741/E811

Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.

Open Access Review

Transcriptomics in amyotrophic lateral sclerosis

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1 Bioinformatics and Human Electrophysiology Laboratory, Department of Informatics, Ionian University, Corfu, Greece
2 National Center for Scientific Research "Demokritos", Institute of Nanoscience and Nanotechnology, Athens, Greece

*Author to whom correspondence should be addressed.

Front. Biosci. (Elite Ed) 2018, 10(1), 103–121; https://doi.org/10.2741/E811
Published: 1 January 2018
(This article belongs to the Special Issue Electrophysiology from bench to bedside)
Abstract

Amyotrophic lateral sclerosis (ALS) is an adult-onset, incurable neurodegenerative disease characterized by the selective death of upper and lowers motor neurons in the spinal cord, brainstem and motor cortex, which ultimately leads to paralysis and death within 2-3 years of onset. ALS is poorly understood, although multiple studies have been proposed to explain the pathophysiological mechanisms of the disorder. The development of microarray technology, for simultaneous analysis of the transcriptional expression of thousands of genes, has provided new possibilities to get better insights into the pathogenesis of ALS, and most important, potential new candidate targets for novel treatments. The present review illustrates current evidences from transcriptomic studies in animal models and human samples, related to ALS pathogenesis in parallel to molecular targets associated with the disease progression. Additionally, alteration of RNA metabolism was identified as a major dysregulated pathway in ALS and via this study, new insights into the contribution of altered transcriptional profiles of microRNAs and ALS-associated ribosomal binding proteins have been investigated, in an effort to understand the functional consequences of widespread RNA dysregulation in the disease’s pathological mechanism.

Keywords
Neurodegenerative disease
Microarray technology
Transcriptomic studies
Mitochondrial stress
ROS production
RNA metabolism
Review
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