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Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70  chaperones are targetted to TOM20-TOM22 receptor complexes.
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Alternative mitochondrial quality control mediated by extracellular release.

Chi-Jing Choong1, Tatsusada Okuno1, Kensuke Ikenaka1

  • 1Department of Neurology, Osaka University Graduate School of Medicine, Suita, Japan.

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|November 21, 2020
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Summary

Cells release mitochondria extracellularly as a quality control mechanism, distinct from mitophagy. This pathway is impaired in Parkinson's disease patients with PRKN mutations.

Keywords:
MitochondriaParkinson diseasemitochondrial quality controlmitophagyparkin

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Area of Science:

  • Cell Biology
  • Mitochondrial Biology
  • Neuroscience

Background:

  • Mitochondrial quality control is vital for cellular health.
  • Mitophagy is the primary known pathway for removing damaged mitochondria.
  • The role of extracellular release in mitochondrial quality control remains largely unexplored.

Purpose of the Study:

  • To investigate an alternative mitochondrial quality control pathway involving extracellular release.
  • To determine the relationship between extracellular mitochondria release and mitophagy.
  • To examine the clinical relevance of extracellular mitochondria release in Parkinson's disease.

Main Methods:

  • Time-lapse confocal imaging of fluorescent-labeled mitochondria.
  • Correlative light-electron microscopy to characterize extracellular mitochondria.
  • Induction of mitochondrial stress using rotenone and CCCP.
  • Genetic manipulation (overexpression and knockdown) of key mitophagy regulators (PRKN, BNIP3, BNIP3L/NIX).
  • Analysis of mitochondrial protein levels in mouse sera and human patient samples (fibroblasts, CSF).

Main Results:

  • Cells were observed releasing mitochondria into the extracellular space, often in free form.
  • Mitochondrial dysfunction induced by rotenone/CCCP increased extracellular release of damaged mitochondria.
  • PRKN (parkin) overexpression suppressed extracellular release, while knockdown exacerbated it.
  • BNIP3 and BNIP3L/NIX also suppressed extracellular release, suggesting interplay with mitophagy.
  • Autophagy-deficient cells showed increased extracellular mitochondria release.
  • Elevated mitochondrial proteins were found in PRKN-deficient mouse sera.
  • Parkinson's disease patients with loss-of-function PRKN mutations exhibited increased extracellular mitochondria in fibroblasts and CSF.

Conclusions:

  • Extracellular mitochondria release represents a distinct, yet comparable, mitochondrial quality control pathway to mitophagy.
  • Perturbation of the mitophagy pathway can lead to increased mitochondria expulsion.
  • This pathway is implicated in the pathogenesis of Parkinson's disease, particularly in patients with PRKN mutations.