The peripheral nervous system in amyotrophic lateral sclerosis: Opportunities for translational research

Francesco Gentile, Stefania Scarlino, Yuri Matteo Falzone, Christian Lunetta, Lucio Tremolizzo, Angelo Quattrini, Nilo Riva

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish
Article number601
JournalFrontiers in Neuroscience
Volume13
Issue numberJUN
DOIs
Publication statusPublished - Jan 1 2019

Keywords

  • CMT
  • Distal SMA
  • Genetics
  • Hereditary neuropathy
  • Lower motor neuron syndrome
  • Motor neuron disease
  • Nerve
  • Neuropathy

ASJC Scopus subject areas

  • Neuroscience(all)

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