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Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers
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Mitochondrial function in neuronal cells depends on p97/VCP/Cdc48-mediated quality control.

Lei Fang1, Charles Hemion1, Ana C Pinho Ferreira Bento1

  • 1Department of Biomedicine, University of Basel Basel, Switzerland.

Frontiers in Cellular Neuroscience
|February 21, 2015
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The AAA-ATPase p97 protein is crucial for maintaining mitochondrial proteostasis and function in neurons. Its dysfunction leads to mitochondrial fragmentation and oxidative stress, impacting neuronal survival.

Keywords:
mitochondrianeuronal cellsp97/VCPquality control

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Area of Science:

  • Cell Biology
  • Neuroscience
  • Biochemistry

Background:

  • Mitochondrial function is vital for neuronal survival and neuroprotection.
  • Outer mitochondrial membrane associated degradation (OMMAD) maintains mitochondrial proteostasis, mitophagy, and cell death pathways.
  • The AAA-ATPase p97/VCP/Cdc48 is implicated in OMMAD, acting as a retrotranslocase for damaged mitochondrial proteins.

Purpose of the Study:

  • To investigate the role of p97 in maintaining mitochondrial function and proteostasis in neuronal cells.
  • To determine the impact of p97 inactivation on mitochondrial integrity under normal and stress conditions.

Main Methods:

  • Utilized SH-SY5Y cells expressing wild-type or dominant-negative p97(QQ).
  • Treated cells with mitochondrial toxins (rotenone, 6-OHDA, Aβ-peptide) to induce mitochondrial dysfunction.
  • Assessed mitochondrial fragmentation, membrane potential, reactive oxygen species (ROS) production, and protein oxidation.

Main Results:

  • p97 inactivation significantly increased mitochondrial fragmentation under both normal and stress conditions.
  • Inactivation of p97 led to loss of mitochondrial membrane potential and elevated ROS production, exacerbated by stress.
  • Accumulation of oxidatively-damaged proteins on mitochondria was observed upon p97 inactivation, indicating impaired proteostasis.

Conclusions:

  • p97 plays a critical role in maintaining mitochondrial proteostasis and function in neuronal cells.
  • Dysfunction of p97 severely impacts mitochondrial integrity, contributing to neuronal vulnerability.
  • This study provides the first evidence for p97 as a key component of mitochondrial proteostasis in neurons.