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Multifunctional Mitochondrial AAA Proteases

Mitochondria perform numerous functions necessary for the survival of eukaryotic cells. These activities are coordinated by a diverse complement of proteins encoded in both the nuclear and mitochondrial genomes that must be properly organized and maintained. Misregulation of mitochondrial proteostas...

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Autor principal: Glynn, Steven E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5438985/
https://www.ncbi.nlm.nih.gov/pubmed/28589125
http://dx.doi.org/10.3389/fmolb.2017.00034
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author Glynn, Steven E.
author_facet Glynn, Steven E.
author_sort Glynn, Steven E.
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description Mitochondria perform numerous functions necessary for the survival of eukaryotic cells. These activities are coordinated by a diverse complement of proteins encoded in both the nuclear and mitochondrial genomes that must be properly organized and maintained. Misregulation of mitochondrial proteostasis impairs organellar function and can result in the development of severe human diseases. ATP-driven AAA+ proteins play crucial roles in preserving mitochondrial activity by removing and remodeling protein molecules in accordance with the needs of the cell. Two mitochondrial AAA proteases, i-AAA and m-AAA, are anchored to either face of the mitochondrial inner membrane, where they engage and process an array of substrates to impact protein biogenesis, quality control, and the regulation of key metabolic pathways. The functionality of these proteases is extended through multiple substrate-dependent modes of action, including complete degradation, partial processing, or dislocation from the membrane without proteolysis. This review discusses recent advances made toward elucidating the mechanisms of substrate recognition, handling, and degradation that allow these versatile proteases to control diverse activities in this multifunctional organelle.
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spelling pubmed-54389852017-06-06 Multifunctional Mitochondrial AAA Proteases Glynn, Steven E. Front Mol Biosci Molecular Biosciences Mitochondria perform numerous functions necessary for the survival of eukaryotic cells. These activities are coordinated by a diverse complement of proteins encoded in both the nuclear and mitochondrial genomes that must be properly organized and maintained. Misregulation of mitochondrial proteostasis impairs organellar function and can result in the development of severe human diseases. ATP-driven AAA+ proteins play crucial roles in preserving mitochondrial activity by removing and remodeling protein molecules in accordance with the needs of the cell. Two mitochondrial AAA proteases, i-AAA and m-AAA, are anchored to either face of the mitochondrial inner membrane, where they engage and process an array of substrates to impact protein biogenesis, quality control, and the regulation of key metabolic pathways. The functionality of these proteases is extended through multiple substrate-dependent modes of action, including complete degradation, partial processing, or dislocation from the membrane without proteolysis. This review discusses recent advances made toward elucidating the mechanisms of substrate recognition, handling, and degradation that allow these versatile proteases to control diverse activities in this multifunctional organelle. Frontiers Media S.A. 2017-05-22 /pmc/articles/PMC5438985/ /pubmed/28589125 http://dx.doi.org/10.3389/fmolb.2017.00034 Text en Copyright © 2017 Glynn. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Molecular Biosciences
Glynn, Steven E.
Multifunctional Mitochondrial AAA Proteases
title Multifunctional Mitochondrial AAA Proteases
title_full Multifunctional Mitochondrial AAA Proteases
title_fullStr Multifunctional Mitochondrial AAA Proteases
title_full_unstemmed Multifunctional Mitochondrial AAA Proteases
title_short Multifunctional Mitochondrial AAA Proteases
title_sort multifunctional mitochondrial aaa proteases
topic Molecular Biosciences
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5438985/
https://www.ncbi.nlm.nih.gov/pubmed/28589125
http://dx.doi.org/10.3389/fmolb.2017.00034
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