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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...

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Autores principales: 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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2016
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.
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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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