AUTHOR=Gentile Francesco , Scarlino Stefania , Falzone Yuri Matteo , Lunetta Christian , Tremolizzo Lucio , Quattrini Angelo , Riva Nilo TITLE=The Peripheral Nervous System in Amyotrophic Lateral Sclerosis: Opportunities for Translational Research JOURNAL=Frontiers in Neuroscience VOLUME=13 YEAR=2019 URL=https://www.frontiersin.org/journals/neuroscience/articles/10.3389/fnins.2019.00601 DOI=10.3389/fnins.2019.00601 ISSN=1662-453X ABSTRACT=

Although amyotrophic lateral sclerosis (ALS) has been considered as a disorder of the motor neuron (MN) cell body, recent evidences show the non-cell-autonomous pathogenic nature of the disease. Axonal degeneration, loss of peripheral axons and destruction of nerve terminals are early events in the disease pathogenic cascade, anticipating MN degeneration, and the onset of clinical symptoms. Therefore, although ALS and peripheral axonal neuropathies should be differentiated in clinical practice, they also share damage to common molecular pathways, including axonal transport, RNA metabolism and proteostasis. Thus, an extensive evaluation of the molecular events occurring in the peripheral nervous system (PNS) could be fundamental to understand the pathogenic mechanisms of ALS, favoring the discovery of potential disease biomarkers, and new therapeutic targets.