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Diseases 2017, 5(1), 1;

Mitochondrial Dynamics in Mitochondrial Diseases

Centro Andaluz de Biología del Desarrollo (CABD-CSIC-Universidad Pablo de Olavide), and Centro de Investigación Biomédica en Red Enfermedades Raras, Instituto de Salud Carlos III, Sevilla 41013, Spain
Author to whom correspondence should be addressed.
Academic Editor: Pedro Bullon
Received: 14 November 2016 / Revised: 13 December 2016 / Accepted: 21 December 2016 / Published: 23 December 2016
(This article belongs to the Special Issue Mitochondria and Mitophagia in Rare Diseases)
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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. View Full-Text
Keywords: mitochondrial disease; mitochondrial dynamics; mitophagy; mitochondrial fusion; mitocondrial fission mitochondrial disease; mitochondrial dynamics; mitophagy; mitochondrial fusion; mitocondrial fission

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Suárez-Rivero, J.M.; Villanueva-Paz, M.; de la Cruz-Ojeda, P.; de la Mata, M.; Cotán, D.; Oropesa-Ávila, M.; de Lavera, I.; Álvarez-Córdoba, M.; Luzón-Hidalgo, R.; Sánchez-Alcázar, J.A. Mitochondrial Dynamics in Mitochondrial Diseases. Diseases 2017, 5, 1.

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