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Molecular Insights into Mitochondrial Protein Translocation and Human Disease
In human mitochondria, mtDNA encodes for only 13 proteins, all components of the OXPHOS system. The rest of the mitochondrial components, which make up approximately 99% of its proteome, are encoded in the nuclear genome, synthesized in cytosolic ribosomes and imported into mitochondria. Different i...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8305315/ https://www.ncbi.nlm.nih.gov/pubmed/34356047 http://dx.doi.org/10.3390/genes12071031 |
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author | Ruiz-Pesini, Eduardo Montoya, Julio Pacheu-Grau, David |
author_facet | Ruiz-Pesini, Eduardo Montoya, Julio Pacheu-Grau, David |
author_sort | Ruiz-Pesini, Eduardo |
collection | PubMed |
description | In human mitochondria, mtDNA encodes for only 13 proteins, all components of the OXPHOS system. The rest of the mitochondrial components, which make up approximately 99% of its proteome, are encoded in the nuclear genome, synthesized in cytosolic ribosomes and imported into mitochondria. Different import machineries translocate mitochondrial precursors, depending on their nature and the final destination inside the organelle. The proper and coordinated function of these molecular pathways is critical for mitochondrial homeostasis. Here, we will review molecular details about these pathways, which components have been linked to human disease and future perspectives on the field to expand the genetic landscape of mitochondrial diseases. |
format | Online Article Text |
id | pubmed-8305315 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-83053152021-07-25 Molecular Insights into Mitochondrial Protein Translocation and Human Disease Ruiz-Pesini, Eduardo Montoya, Julio Pacheu-Grau, David Genes (Basel) Review In human mitochondria, mtDNA encodes for only 13 proteins, all components of the OXPHOS system. The rest of the mitochondrial components, which make up approximately 99% of its proteome, are encoded in the nuclear genome, synthesized in cytosolic ribosomes and imported into mitochondria. Different import machineries translocate mitochondrial precursors, depending on their nature and the final destination inside the organelle. The proper and coordinated function of these molecular pathways is critical for mitochondrial homeostasis. Here, we will review molecular details about these pathways, which components have been linked to human disease and future perspectives on the field to expand the genetic landscape of mitochondrial diseases. MDPI 2021-07-01 /pmc/articles/PMC8305315/ /pubmed/34356047 http://dx.doi.org/10.3390/genes12071031 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Ruiz-Pesini, Eduardo Montoya, Julio Pacheu-Grau, David Molecular Insights into Mitochondrial Protein Translocation and Human Disease |
title | Molecular Insights into Mitochondrial Protein Translocation and Human Disease |
title_full | Molecular Insights into Mitochondrial Protein Translocation and Human Disease |
title_fullStr | Molecular Insights into Mitochondrial Protein Translocation and Human Disease |
title_full_unstemmed | Molecular Insights into Mitochondrial Protein Translocation and Human Disease |
title_short | Molecular Insights into Mitochondrial Protein Translocation and Human Disease |
title_sort | molecular insights into mitochondrial protein translocation and human disease |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8305315/ https://www.ncbi.nlm.nih.gov/pubmed/34356047 http://dx.doi.org/10.3390/genes12071031 |
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