Citation

  • Authors: Durcan, T. M., Tang, M. Y., Perusse, J. R., Dashti, E. A., Aguileta, M. A., McLelland, G. L., Gros, P., Shaler, T. A., Faubert, D., Coulombe, B., Fon, E. A.
  • Year: 2014
  • Journal: EMBO J 33 2473-91
  • Applications: in vitro / DNA, siRNA and DNA cotransfection / jetPRIME
  • Cell types:
    1. Name: HEK-293T
      Description: Human embryonic kidney Fibroblast
      Known as: HEK293T, 293T
    2. Name: HeLa
      Description: Human cervix epitheloid carcinoma cells
    3. Name: SH-SY5Y
      Description: Human neuroblastoma cells
      Known as:
    4. Name: U-2 OS
      Description: Human bone osteosarcoma
      Known as: U2OS

Abstract

Mutations in the Park2 gene, encoding the E3 ubiquitin-ligase parkin, are responsible for a familial form of Parkinson's disease (PD). Parkin-mediated ubiquitination is critical for the efficient elimination of depolarized dysfunctional mitochondria by autophagy (mitophagy). As damaged mitochondria are a major source of toxic reactive oxygen species within the cell, this pathway is believed to be highly relevant to the pathogenesis of PD. Little is known about how parkin-mediated ubiquitination is regulated during mitophagy or about the nature of the ubiquitin conjugates involved. We report here that USP8/UBPY, a deubiquitinating enzyme not previously implicated in mitochondrial quality control, is critical for parkin-mediated mitophagy. USP8 preferentially removes non-canonical K6-linked ubiquitin chains from parkin, a process required for the efficient recruitment of parkin to depolarized mitochondria and for their subsequent elimination by mitophagy. This work uncovers a novel role for USP8-mediated deubiquitination of K6-linked ubiquitin conjugates from parkin in mitochondrial quality control.

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