Stress Granules and ALS: A Case of Causation or Correlation?

Adv Neurobiol. 2018:20:173-212. doi: 10.1007/978-3-319-89689-2_7.

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by cytoplasmic protein aggregates within motor neurons. These aggregates are linked to ALS pathogenesis. Recent evidence has suggested that stress granules may aid the formation of ALS protein aggregates. Here, we summarize current understanding of stress granules, focusing on assembly and clearance. We also assess the evidence linking alterations in stress granule formation and dynamics to ALS protein aggregates and disease pathology.

Keywords: ALS; Autophagy; C9ORF72; Chaperones; Cytoskeleton; FUS; SOD1; Stress granules; TDP-43; mRNA.

Publication types

  • Review

MeSH terms

  • Amyotrophic Lateral Sclerosis / metabolism*
  • Autophagy / physiology
  • Cytoplasmic Granules / metabolism*
  • Humans
  • Motor Neurons / metabolism*
  • RNA-Binding Protein FUS / metabolism

Substances

  • RNA-Binding Protein FUS