Mitochondria and Mitophagia in Rare Diseases

A special issue of Diseases (ISSN 2079-9721). This special issue belongs to the section "Rare Syndrome".

Deadline for manuscript submissions: closed (30 September 2016) | Viewed by 11263

Special Issue Editor


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Guest Editor
Department of Periodontology, Dental School, University of Sevilla, C/Avicena s.n., Sevilla 41009, Spain
Interests: oral pathology; systemic and rare diseases in oral pathology; periodontal diseases/periodontitis; cardiovascular disease, diabetes and low birth weight in periodontitis; oxidative stress; nutrition; aging; mitochondrial disfunction/diseases

Special Issue Information

Dear Colleagues,

The main objective to improve our success in the treatment of rare diseases is to understand the pathogenic mechanisms involved. For the last 10 years, a great deal of new data have been published, especially in the field of cell metabolism and energy production. Mitochondria are key organelles involved in ATP synthesis through the electron transport chain. They are under high stress and hazard, and it is essential to maintain them in a healthy condition with autophagy/mitophagy processes. The inheritance of mitADN and nADN, which control energy production, are involved in rare diseases. We need to update the role of the mitochondrial dysfunction and genome. Mitochondrial diseases are a clinically heterogeneous group of disorders that arise as a result of dysfunction of the mitochondrial respiratory chain. Inherited metabolic disorders involved the autophagy pathway and are an emerging, novel, and diverse group of multisystem diseases. It is essential to have testing tools needed to diagnose the mitochondrial mechanisms involved in rare diseases. All of them can improve our treatment results and give new hope to families.

Prof. Dr. Pedro Bullon
Guest Editor

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Keywords

  • energy production and mitochondria
  • autophagy and mitophagy
  • mitochondrial inheritance
  • mitochondrial dysfunction in rare diseases
  • mitochondrial genome in rare diseases
  • mitochondrial disorders
  • inherited metabolic disorders
  • diagnosis/testing of mitochondrial disorders in rare diseases
  • gene mitochondrial therapies

Published Papers (1 paper)

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Review
Mitochondrial Dynamics in Mitochondrial Diseases
by Juan M. Suárez-Rivero, Marina Villanueva-Paz, Patricia De la Cruz-Ojeda, Mario De la Mata, David Cotán, Manuel Oropesa-Ávila, Isabel De Lavera, Mónica Álvarez-Córdoba, Raquel Luzón-Hidalgo and José A. Sánchez-Alcázar
Diseases 2017, 5(1), 1; https://doi.org/10.3390/diseases5010001 - 23 Dec 2016
Cited by 114 | Viewed by 10683
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Mitochondria and Mitophagia in Rare Diseases)
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