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Free keywords:
Adenosine Triphosphate/metabolism
Animals
Cellular Senescence
GTP Phosphohydrolases/metabolism/physiology
Homeostasis
Humans
Mitochondria/metabolism/*physiology/ultrastructure
Mitochondrial Proteins/*metabolism/physiology
Models, Biological
Molecular Chaperones/*metabolism/physiology
Peptide Hydrolases/metabolism/physiology
Proteasome Endopeptidase Complex/metabolism/physiology
Protein Folding
Ubiquitin/metabolism
Abstract:
A decline in mitochondrial activity has been associated with aging and is a hallmark of many neurological diseases. Surveillance mechanisms acting at the molecular, organellar, and cellular level monitor mitochondrial integrity and ensure the maintenance of mitochondrial proteostasis. Here we will review the central role of mitochondrial chaperones and proteases, the cytosolic ubiquitin-proteasome system, and the mitochondrial unfolded response in this interconnected quality control network, highlighting the dual function of some proteases in protein quality control within the organelle and for the regulation of mitochondrial fusion and mitophagy.
