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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
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Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
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Mitochondria are double-membrane organelles of the eukaryotes involved in cellular metabolism, signaling, ATP synthesis, and programmed cell death.  Each of these processes requires specific proteins and enzymes that must be correctly sorted to the right mitochondrial subcompartment for the proper functioning of the organelle.
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Failure to Guard: Mitochondrial Protein Quality Control in Cancer.

Joseph E Friedlander1, Ning Shen1,2, Aozhuo Zeng1

  • 1Department of Pharmacology and Experimental Therapeutics, Boston University School of Medicine, Boston, MA 02118, USA.

International Journal of Molecular Sciences
|August 7, 2021
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Summary

Mitochondrial protein quality control (MPQC) maintains cellular health by safeguarding proteins within mitochondria. Dysfunctional MPQC is linked to diseases like cancer, highlighting its therapeutic potential.

Keywords:
MPQCcancerchaperonemitochondriaoncogeneproteaseproteostasistherapeutic targetingtumor suppressortumorigenesis

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Area of Science:

  • Cell Biology
  • Molecular Biology
  • Biochemistry

Background:

  • Mitochondria are vital organelles involved in energy production, metabolism, and signaling.
  • Maintaining mitochondrial proteostasis is essential for cellular function.
  • Mitochondrial protein quality control (MPQC) is a critical surveillance system.

Purpose of the Study:

  • To review key pathways and regulators involved in MPQC.
  • To explore the link between MPQC dysfunction and human diseases, particularly cancer.
  • To summarize recent therapeutic strategies targeting MPQC in cancer treatment.

Main Methods:

  • Review of existing literature on MPQC pathways.
  • Analysis of MPQC components in relation to disease mechanisms.
  • Summary of emerging therapeutic approaches.

Main Results:

  • MPQC involves pathways like protein translocation-associated degradation, stress responses, chaperones, and proteases.
  • Deregulated MPQC leads to proteotoxicity and mitochondrial dysfunction.
  • Alterations in MPQC components are implicated in tumorigenesis, acting as drivers or suppressors.

Conclusions:

  • MPQC is crucial for maintaining mitochondrial health and integrity.
  • MPQC dysfunction contributes significantly to the pathogenesis of various diseases, including cancer.
  • Targeting MPQC alterations presents a promising avenue for developing novel anti-cancer therapies.