The Cellular and Molecular Signature of ALS in Muscle

Research output: Contribution to journalReview articlepeer-review

Abstract

Amyotrophic lateral sclerosis is a disease affecting upper and lower motor neurons. Although motor neuron death is the core event of ALS pathology, it is increasingly recognized that other tissues and cell types are affected in the disease, making potentially major contributions to the occurrence and progression of pathology. We review here the known cellular and molecular characteristics of muscle tissue affected by ALS. Evidence of toxicity in skeletal muscle tissue is considered, including metabolic dysfunctions, impaired proteostasis, and deficits in muscle regeneration and RNA metabolism. The role of muscle as a secretory organ, and effects on the skeletal muscle secretome are also covered, including the increase in secretion of toxic factors or decrease in essential factors that have consequences for neuronal function and survival.
Original languageEnglish
Article number1868
Pages (from-to)1-16
Number of pages16
JournalJournal of Personalized Medicine
Volume12
Issue number11
Early online date8 Nov 2022
DOIs
Publication statusPublished online - 8 Nov 2022

Bibliographical note

Publisher Copyright:
© 2022 by the authors.

Keywords

  • muscle regeneration
  • motor neurone disease
  • muscle secretome
  • muscle metabolism
  • muscle-nerve communication
  • neuromuscular junction

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