Mitochondrial Quality Control and Cellular Proteostasis: Two Sides of the Same Coin

Frontiers in Physiology
Justin M Quiles, Åsa B Gustafsson

Abstract

Mitochondrial dysfunction is a hallmark of cardiac pathophysiology. Defects in mitochondrial performance disrupt contractile function, overwhelm myocytes with reactive oxygen species (ROS), and transform these cellular powerhouses into pro-death organelles. Thus, quality control (QC) pathways aimed at identifying and removing damaged mitochondrial proteins, components, or entire mitochondria are crucial processes in post-mitotic cells such as cardiac myocytes. Almost all of the mitochondrial proteins are encoded by the nuclear genome and the trafficking of these nuclear-encoded proteins necessitates significant cross-talk with the cytosolic protein QC machinery to ensure that only functional proteins are delivered to the mitochondria. Within the organelle, mitochondria contain their own protein QC system consisting of chaperones and proteases. This system represents another level of QC to promote mitochondrial protein folding and prevent aggregation. If this system is overwhelmed, a conserved transcriptional response known as the mitochondrial unfolded protein response is activated to increase the expression of proteins involved in restoring mitochondrial proteostasis. If the mitochondrion is beyond repair, the entire organelle...Continue Reading

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Citations

Nov 30, 2020·FEBS Letters·Lena KrämerJohannes M Herrmann
Dec 18, 2020·Cardiovascular Research·Toshiro SaitoJunichi Sadoshima
Jan 9, 2021·Biochimica Et Biophysica Acta. Bioenergetics·Karolina Szczepanowska, Aleksandra Trifunovic
Feb 3, 2021·The International Journal of Biochemistry & Cell Biology·Ioannis Smyrnias
Feb 21, 2021·Pharmacological Research : the Official Journal of the Italian Pharmacological Society·Yang ChenBin Shen
Dec 30, 2021·American Journal of Physiology. Cell Physiology·Rachel Y Diao, Åsa B Gustafsson

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Methods Mentioned

BETA
biopsies
ubiquitination
protein folding
nuclear translocation
ubiquitinate

Clinical Trials Mentioned

NCT03727646

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