Epigenetic Mechanisms of Gene Regulation in Amyotrophic Lateral Sclerosis

Advances in Experimental Medicine and Biology
Alba Jimenez-PachecoDavid Pozo

Abstract

Despite being clinically described 150 years ago, the mechanisms underlying amyotrophic lateral sclerosis (ALS) pathogenesis have not yet been fully understood. Studies in both animal models of ALS and human patients reveal a plethora of alterations such as increased glutamate-mediated excitotoxicity, redox stress, increased apoptosis, defective axonal transport, protein-misfolding events, mitochondrial impairment and sustained unregulated immune responses. Regardless of being sporadic or familiar ALS, the final outcome at the cellular level is the death of upper and lower motor neurons, and once diagnosed, ALS is typically lethal within the next 5 years. There are neither clear biomarkers nor therapeutic or disease-modifying treatments for ALS.Accumulating evidence supports the concept that epigenetic-driven modifications, including altered chromatin remodelling events, RNA editing and non-coding RNA molecules, might shed light into the pathogenic mechanisms underlying sporadic/familiar ALS onset and/or severity to facilitate the identification of effective therapies, early diagnosis and potentially early-stage therapeutic interventions to increase the survival outcome of ALS patients.

Citations

Jan 22, 2019·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·R S BedlackGhazaleh Sadri-Vakili
Sep 19, 2018·Alzheimer's & Dementia : Translational Research & Clinical Interventions·Surbhi SharmaMartin R Schiller
Jun 4, 2020·Molecular Neurodegeneration·Rajka Maria LiscicEmanuele Buratti
Feb 6, 2018·Therapeutic Advances in Neurological Disorders·Johannes DorstAnnemarie Huebers
Jun 22, 2018·Annals of Clinical and Translational Neurology·Maite MendiorozIvonne Jericó
Jun 2, 2020·Frontiers in Cellular Neuroscience·Ximena Paez-ColasanteEva L Feldman
Mar 7, 2020·International Journal of Molecular Sciences·Metka Ravnik-Glavač, Damjan Glavač
Dec 8, 2019·Neuron·Tingting WangGraeme W Davis
May 7, 2021·Computational and Mathematical Methods in Medicine·Dingsheng LiuXuefeng Gu
Apr 6, 2020·Brain Research Bulletin·Auderlan M GoisJose R Santos

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