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Mitochondrial Dynamics in Mitochondrial Diseases
Mitochondria are very versatile organelles in continuous fusion and fission processes in response to various cellular signals. Mitochondrial dynamics, including mitochondrial fission/fusion, movements and turnover, are essential for the mitochondrial network quality control. Alterations in mitochond...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5456341/ https://www.ncbi.nlm.nih.gov/pubmed/28933354 http://dx.doi.org/10.3390/diseases5010001 |
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author | Suárez-Rivero, Juan M. Villanueva-Paz, Marina de la Cruz-Ojeda, Patricia de la Mata, Mario Cotán, David Oropesa-Ávila, Manuel de Lavera, Isabel Álvarez-Córdoba, Mónica Luzón-Hidalgo, Raquel Sánchez-Alcázar, José A. |
author_facet | Suárez-Rivero, Juan M. Villanueva-Paz, Marina de la Cruz-Ojeda, Patricia de la Mata, Mario Cotán, David Oropesa-Ávila, Manuel de Lavera, Isabel Álvarez-Córdoba, Mónica Luzón-Hidalgo, Raquel Sánchez-Alcázar, José A. |
author_sort | Suárez-Rivero, Juan M. |
collection | PubMed |
description | Mitochondria are very versatile organelles in continuous fusion and fission processes in response to various cellular signals. Mitochondrial dynamics, including mitochondrial fission/fusion, movements and turnover, are essential for the mitochondrial network quality control. Alterations in mitochondrial dynamics can cause neuropathies such as Charcot-Marie-Tooth disease in which mitochondrial fusion and transport are impaired, or dominant optic atrophy which is caused by a reduced mitochondrial fusion. On the other hand, mitochondrial dysfunction in primary mitochondrial diseases promotes reactive oxygen species production that impairs its own function and dynamics, causing a continuous vicious cycle that aggravates the pathological phenotype. Mitochondrial dynamics provides a new way to understand the pathophysiology of mitochondrial disorders and other diseases related to mitochondria dysfunction such as diabetes, heart failure, or Hungtinton’s disease. The knowledge about mitochondrial dynamics also offers new therapeutics targets in mitochondrial diseases. |
format | Online Article Text |
id | pubmed-5456341 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-54563412017-09-12 Mitochondrial Dynamics in Mitochondrial Diseases Suárez-Rivero, Juan M. Villanueva-Paz, Marina de la Cruz-Ojeda, Patricia de la Mata, Mario Cotán, David Oropesa-Ávila, Manuel de Lavera, Isabel Álvarez-Córdoba, Mónica Luzón-Hidalgo, Raquel Sánchez-Alcázar, José A. Diseases Review Mitochondria are very versatile organelles in continuous fusion and fission processes in response to various cellular signals. Mitochondrial dynamics, including mitochondrial fission/fusion, movements and turnover, are essential for the mitochondrial network quality control. Alterations in mitochondrial dynamics can cause neuropathies such as Charcot-Marie-Tooth disease in which mitochondrial fusion and transport are impaired, or dominant optic atrophy which is caused by a reduced mitochondrial fusion. On the other hand, mitochondrial dysfunction in primary mitochondrial diseases promotes reactive oxygen species production that impairs its own function and dynamics, causing a continuous vicious cycle that aggravates the pathological phenotype. Mitochondrial dynamics provides a new way to understand the pathophysiology of mitochondrial disorders and other diseases related to mitochondria dysfunction such as diabetes, heart failure, or Hungtinton’s disease. The knowledge about mitochondrial dynamics also offers new therapeutics targets in mitochondrial diseases. MDPI 2016-12-23 /pmc/articles/PMC5456341/ /pubmed/28933354 http://dx.doi.org/10.3390/diseases5010001 Text en © 2016 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Suárez-Rivero, Juan M. Villanueva-Paz, Marina de la Cruz-Ojeda, Patricia de la Mata, Mario Cotán, David Oropesa-Ávila, Manuel de Lavera, Isabel Álvarez-Córdoba, Mónica Luzón-Hidalgo, Raquel Sánchez-Alcázar, José A. Mitochondrial Dynamics in Mitochondrial Diseases |
title | Mitochondrial Dynamics in Mitochondrial Diseases |
title_full | Mitochondrial Dynamics in Mitochondrial Diseases |
title_fullStr | Mitochondrial Dynamics in Mitochondrial Diseases |
title_full_unstemmed | Mitochondrial Dynamics in Mitochondrial Diseases |
title_short | Mitochondrial Dynamics in Mitochondrial Diseases |
title_sort | mitochondrial dynamics in mitochondrial diseases |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5456341/ https://www.ncbi.nlm.nih.gov/pubmed/28933354 http://dx.doi.org/10.3390/diseases5010001 |
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