4.5 Article

The basic machineries for mitochondrial protein quality control

Journal

MITOCHONDRION
Volume 50, Issue -, Pages 121-131

Publisher

ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2019.10.003

Keywords

Mitochondria; Proteostasis; Quality control; Proteases; Chaperones; Mitophagy; Aggregates

Funding

  1. Swedish Research Council
  2. Knut and Alice Wallenberg Foundation
  3. Austrian Science Fund FWF [P27183-B24]
  4. Austrian Science Fund (FWF) [P27183] Funding Source: Austrian Science Fund (FWF)

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Mitochondria play pivotal roles in cellular energy metabolism, the synthesis of essential biomolecules and the regulation of cell death and aging. The proper folding, unfolding and degradation of the many proteins active within mitochondria is surveyed by the mitochondrial quality control machineries. Here, we describe the principal components of the mitochondrial quality control system and recent developments in the elucidation of the molecular mechanisms maintaining a functional mitochondrial proteome.

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