Siah3 acts as a physiological mitophagy suppressor that facilitates axonal degeneration

Sci Signal. 2024 Oct 8;17(857):eadn5805. doi: 10.1126/scisignal.adn5805. Epub 2024 Oct 8.

Abstract

Mitophagy eliminates dysfunctional mitochondria, and defects in this cellular housekeeping mechanism are implicated in various age-related diseases. Here, we found that mitophagy suppression by the protein Siah3 promoted developmental axonal remodeling in mice. Siah3-deficient mice displayed increased peripheral sensory innervation. Cultured Siah3-deficient sensory neurons exhibited delays in both axonal degeneration and caspase-3 activation in response to withdrawal of nerve growth factor. Mechanistically, Siah3 was transcriptionally induced by the loss of trophic support and formed a complex with the cytosolic E3 ubiquitin ligase parkin, a core component of mitophagy, in transfected cells. Axons of Siah3-deficient neurons mounted profound mitophagy upon initiation of degeneration but not under basal conditions. Neurons lacking both Siah3 and parkin did not exhibit the delay in trophic deprivation-induced axonal degeneration or the induction of axonal mitophagy that was seen in Siah3-deficient neurons. Our findings reveal that mitophagy regulation acts as a gatekeeper of a physiological axon elimination program.

MeSH terms

  • Animals
  • Axons* / metabolism
  • Axons* / pathology
  • Caspase 3 / genetics
  • Caspase 3 / metabolism
  • Cells, Cultured
  • Humans
  • Mice
  • Mice, Knockout*
  • Mitochondria / genetics
  • Mitochondria / metabolism
  • Mitophagy* / genetics
  • Nerve Degeneration / genetics
  • Nerve Degeneration / metabolism
  • Nerve Degeneration / pathology
  • Nerve Growth Factor / genetics
  • Nerve Growth Factor / metabolism
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism
  • Sensory Receptor Cells / metabolism
  • Sensory Receptor Cells / pathology
  • Ubiquitin-Protein Ligases* / genetics
  • Ubiquitin-Protein Ligases* / metabolism

Substances

  • Ubiquitin-Protein Ligases
  • seven in absentia proteins
  • parkin protein
  • Nuclear Proteins
  • Caspase 3
  • Nerve Growth Factor