Hsc70/Stub1 promotes the removal of individual oxidatively stressed peroxisomes

Nat Commun. 2020 Oct 19;11(1):5267. doi: 10.1038/s41467-020-18942-3.

Abstract

Peroxisomes perform beta-oxidation of branched and very-long chain fatty acids, which leads to the formation of reactive oxygen species (ROS) within the peroxisomal lumen. Peroxisomes are therefore prone to ROS-mediated damages. Here, using light to specifically and acutely induce ROS formation within the peroxisomal lumen, we find that cells individually remove ROS-stressed peroxisomes through ubiquitin-dependent pexophagy. Heat shock protein 70 s mediates the translocation of the ubiquitin E3 ligase Stub1 (STIP1 Homology and U-Box Containing Protein 1) onto oxidatively-stressed peroxisomes to promote their selective ubiquitination and autophagic degradation. Artificially targeting Stub1 to healthy peroxisomes is sufficient to trigger pexophagy, suggesting a key role Stub1 plays in regulating peroxisome quality. We further determine that Stub1 mutants found in Ataxia patients are defective in pexophagy induction. Dysfunctional peroxisomal quality control may therefore contribute to the development of Ataxia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Ataxia / genetics
  • Ataxia / metabolism
  • Ataxia / physiopathology
  • Biological Transport
  • Cell Line
  • HSC70 Heat-Shock Proteins / genetics
  • HSC70 Heat-Shock Proteins / metabolism*
  • Humans
  • Macroautophagy
  • Oxidative Stress*
  • Peroxisomes / genetics
  • Peroxisomes / metabolism*
  • Reactive Oxygen Species / metabolism
  • Ubiquitin-Protein Ligases / genetics
  • Ubiquitin-Protein Ligases / metabolism*
  • Ubiquitination

Substances

  • HSC70 Heat-Shock Proteins
  • HSPA8 protein, human
  • Reactive Oxygen Species
  • STUB1 protein, human
  • Ubiquitin-Protein Ligases