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Aberrant Stress Granule Dynamics and Aggrephagy in ALS Pathogenesis.

Yi ZhangJiayu GuQiming Sun
Published in: Cells (2021)
Stress granules are conserved cytosolic ribonucleoprotein (RNP) compartments that undergo dynamic assembly and disassembly by phase separation in response to stressful conditions. Gene mutations may lead to aberrant phase separation of stress granules eliciting irreversible protein aggregations. A selective autophagy pathway called aggrephagy may partially alleviate the cytotoxicity mediated by these protein aggregates. Cells must perceive when and where the stress granules are transformed into toxic protein aggregates to initiate autophagosomal engulfment for subsequent autolysosomal degradation, therefore, maintaining cellular homeostasis. Indeed, defective aggrephagy has been causally linked to various neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). In this review, we discuss stress granules at the intersection of autophagy and ALS pathogenesis.
Keyphrases
  • amyotrophic lateral sclerosis
  • stress induced
  • cell death
  • signaling pathway
  • endoplasmic reticulum stress
  • induced apoptosis
  • oxidative stress
  • protein protein
  • amino acid
  • transcription factor
  • cell cycle arrest