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

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

Nikita Fernandes  1 , Nichole Eshleman  1 , J Ross Buchan  2
Affiliations
Review

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

Nikita Fernandes et al. Adv Neurobiol. 2018.

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.

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