Targeting autophagy and mitophagy for mitochondrial diseases treatment |
| |
Authors: | Marina Villanueva Paz David Cotán Juan Garrido-Maraver Mario D. Cordero Manuel Oropesa-Ávila Mario de La Mata |
| |
Affiliation: | 1. Centro Andaluz de Biología del Desarrollo (CABD), and Centro de Investigación Biomédica en Red: Enfermedades Raras, Instituto de Salud Carlos III, Universidad Pablo de Olavide-Consejo Superior de Investigaciones Científicas, Carretera de Utrera Km 1, Sevilla 41013, Spain;2. Facultad de Odontología, Universidad de Sevilla, Sevilla 41009, Spain |
| |
Abstract: | Introduction: Mitochondrial diseases are a group of rare genetic diseases with complex and heterogeneous origins which manifest a great variety of phenotypes. Disruption of the oxidative phosphorylation system is the main cause of pathogenicity in mitochondrial diseases since it causes accumulation of reactive oxygen species (ROS) and ATP depletion.Areas covered: Current evidences support the main protective role of autophagy and mitophagy in mitochondrial diseases and other diseases associated with mitochondrial dysfunction.Expert Opinion: The use of autophagy and/or mitophagy inducers may allow a novel strategy for improving mitochondrial function for both mitochondrial diseases and other diseases with altered mitochondrial metabolism. However, a deeper investigation of the molecular mechanisms behind mitophagy and mitochondrial biogenesis is needed in order to safely modulate these processes. In the coming years, we will also see an increase in awareness of mitochondrial dynamics modulation that will allow the therapeutic use of new drugs for improving mitochondrial function in a great variety of mitochondrial disorders. |
| |
Keywords: | AICAR AMPK autophagy coenzyme Q10 heteroplasmy mitochondrial dynamics mitophagy mTOR Parkin rapamycin ROS |
|
|