Where and why modeling amyotrophic lateral sclerosis

Francesco Liguori, Susanna Amadio, Cinzia Volonté

Research output: Contribution to journalReview articlepeer-review


Over the years, researchers have leveraged a host of different in vivo models in order to dissect amyotrophic lateral sclerosis (ALS), a neurodegenerative/neuroinflammatory disease that is heterogeneous in its clinical presentation and is multigenic, multifactorial and non‐cell autonomous. These models include both vertebrates and invertebrates such as yeast, worms, flies, zebrafish, mice, rats, guinea pigs, dogs and, more recently, non‐human primates. Despite their obvious differences and peculiarities, only the concurrent and comparative analysis of these various systems will allow the untangling of the causes and mechanisms of ALS for finally obtaining new efficacious therapeutics. However, harnessing these powerful organisms poses numerous challenges. In this context, we present here an updated and comprehensive review of how eukaryotic unicellular and multicellular organisms that reproduce a few of the main clinical features of the disease have helped in ALS research to dissect the pathological pathways of the disease insurgence and progression. We describe common features as well as discrepancies among these models, highlighting new insights and emerging roles for experimental organisms in ALS.

Original languageEnglish
Article number3977
JournalInternational Journal of Molecular Sciences
Issue number8
Publication statusPublished - Apr 2 2021


  • Amyotrophic lateral sclerosis
  • Animal modeling
  • Caenorhabditis elegans
  • Canines
  • Danio rerio
  • Drosophila melanogaster
  • Non‐human primates
  • Rodents
  • Saccharomyces cerevisiae

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry


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