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Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones

Mitochondria have a central role in cellular metabolism; they are responsible for the biosynthesis of amino acids, lipids, iron–sulphur clusters and regulate apoptosis. About 99% of mitochondrial proteins are encoded by nuclear genes, so the biogenesis of mitochondria heavily depends on protein impo...

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Autores principales: Dimogkioka, Anna-Roza, Lees, Jamie, Lacko, Erik, Tokatlidis, Kostas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Royal Society of Chemistry 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9041937/
https://www.ncbi.nlm.nih.gov/pubmed/35495482
http://dx.doi.org/10.1039/d1ra04497d
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author Dimogkioka, Anna-Roza
Lees, Jamie
Lacko, Erik
Tokatlidis, Kostas
author_facet Dimogkioka, Anna-Roza
Lees, Jamie
Lacko, Erik
Tokatlidis, Kostas
author_sort Dimogkioka, Anna-Roza
collection PubMed
description Mitochondria have a central role in cellular metabolism; they are responsible for the biosynthesis of amino acids, lipids, iron–sulphur clusters and regulate apoptosis. About 99% of mitochondrial proteins are encoded by nuclear genes, so the biogenesis of mitochondria heavily depends on protein import pathways into the organelle. An intricate system of well-studied import machinery facilitates the import of mitochondrial proteins. In addition, folding of the newly synthesized proteins takes place in a busy environment. A system of folding helper proteins, molecular chaperones and co-chaperones, are present to maintain proper conformation and thus avoid protein aggregation and premature damage. The components of the import machinery are well characterised, but the targeting signals and how they are recognised and decoded remains in some cases unclear. Here we provide some detail on the types of targeting signals involved in the protein import process. Furthermore, we discuss the very elaborate chaperone systems of the intermembrane space that are needed to overcome the particular challenges for the folding process in this compartment. The mechanisms that sustain productive folding in the face of aggregation and damage in mitochondria are critical components of the stress response and play an important role in cell homeostasis.
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spelling pubmed-90419372022-04-28 Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones Dimogkioka, Anna-Roza Lees, Jamie Lacko, Erik Tokatlidis, Kostas RSC Adv Chemistry Mitochondria have a central role in cellular metabolism; they are responsible for the biosynthesis of amino acids, lipids, iron–sulphur clusters and regulate apoptosis. About 99% of mitochondrial proteins are encoded by nuclear genes, so the biogenesis of mitochondria heavily depends on protein import pathways into the organelle. An intricate system of well-studied import machinery facilitates the import of mitochondrial proteins. In addition, folding of the newly synthesized proteins takes place in a busy environment. A system of folding helper proteins, molecular chaperones and co-chaperones, are present to maintain proper conformation and thus avoid protein aggregation and premature damage. The components of the import machinery are well characterised, but the targeting signals and how they are recognised and decoded remains in some cases unclear. Here we provide some detail on the types of targeting signals involved in the protein import process. Furthermore, we discuss the very elaborate chaperone systems of the intermembrane space that are needed to overcome the particular challenges for the folding process in this compartment. The mechanisms that sustain productive folding in the face of aggregation and damage in mitochondria are critical components of the stress response and play an important role in cell homeostasis. The Royal Society of Chemistry 2021-10-01 /pmc/articles/PMC9041937/ /pubmed/35495482 http://dx.doi.org/10.1039/d1ra04497d Text en This journal is © The Royal Society of Chemistry https://creativecommons.org/licenses/by/3.0/
spellingShingle Chemistry
Dimogkioka, Anna-Roza
Lees, Jamie
Lacko, Erik
Tokatlidis, Kostas
Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones
title Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones
title_full Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones
title_fullStr Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones
title_full_unstemmed Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones
title_short Protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones
title_sort protein import in mitochondria biogenesis: guided by targeting signals and sustained by dedicated chaperones
topic Chemistry
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9041937/
https://www.ncbi.nlm.nih.gov/pubmed/35495482
http://dx.doi.org/10.1039/d1ra04497d
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