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Lenkiewicz AM, Krakowczyk M, Bragoszewski P. Cytosolic Quality Control of Mitochondrial Protein Precursors-The Early Stages of the Organelle Biogenesis. Int J Mol Sci. 2021;23(1)doi: 10.3390/ijms23010007.
Lenkiewicz, A. M., Krakowczyk, M., & Bragoszewski, P. (2021). Cytosolic Quality Control of Mitochondrial Protein Precursors-The Early Stages of the Organelle Biogenesis. International journal of molecular sciences, 23(1), . https://doi.org/10.3390/ijms23010007
Lenkiewicz, Anna M, et al. "Cytosolic Quality Control of Mitochondrial Protein Precursors-The Early Stages of the Organelle Biogenesis." International journal of molecular sciences vol. 23,1 (2021). doi: https://doi.org/10.3390/ijms23010007
Lenkiewicz AM, Krakowczyk M, Bragoszewski P. Cytosolic Quality Control of Mitochondrial Protein Precursors-The Early Stages of the Organelle Biogenesis. Int J Mol Sci. 2021 Dec 21;23(1). doi: 10.3390/ijms23010007. PMID: 35008433.
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Int J Mol Sci. 2021 Dec 21;23(1). doi: 10.3390/ijms23010007.

Cytosolic Quality Control of Mitochondrial Protein Precursors-The Early Stages of the Organelle Biogenesis.

International journal of molecular sciences

Anna M Lenkiewicz, Magda Krakowczyk, Piotr Bragoszewski

Affiliations

  1. Laboratory of Protein Homeostasis, Nencki Institute of Experimental Biology, Polish Academy of Sciences, 3 Pasteur Street, 02-093 Warsaw, Poland.

PMID: 35008433 DOI: 10.3390/ijms23010007

Abstract

With few exceptions, proteins that constitute the proteome of mitochondria originate outside of this organelle in precursor forms. Such protein precursors follow dedicated transportation paths to reach specific parts of mitochondria, where they complete their maturation and perform their functions. Mitochondrial precursor targeting and import pathways are essential to maintain proper mitochondrial function and cell survival, thus are tightly controlled at each stage. Mechanisms that sustain protein homeostasis of the cytosol play a vital role in the quality control of proteins targeted to the organelle. Starting from their synthesis, precursors are constantly chaperoned and guided to reduce the risk of premature folding, erroneous interactions, or protein damage. The ubiquitin-proteasome system provides proteolytic control that is not restricted to defective proteins but also regulates the supply of precursors to the organelle. Recent discoveries provide evidence that stress caused by the mislocalization of mitochondrial proteins may contribute to disease development. Precursors are not only subject to regulation but also modulate cytosolic machinery. Here we provide an overview of the cellular pathways that are involved in precursor maintenance and guidance at the early cytosolic stages of mitochondrial biogenesis. Moreover, we follow the circumstances in which mitochondrial protein import deregulation disturbs the cellular balance, carefully looking for rescue paths that can restore proteostasis.

Keywords: mitochondrial biogenesis; molecular chaperone; proteasome; protein degradation; protein precursor; protein transport; proteostasis; quality control; ubiquitin

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