Mitophagy in neurodegenerative diseases. (July 2018)

• Record Type: Journal Article
• Authors: Rodolfo, Carlo; Campello, Silvia; Cecconi, Francesco
• Is Part Of: Neurochemistry international. Volume 117(2018)
• Page Start: 156
• Page End: 166
• Publication Date: 2018-07
• Subjects: Neurochemistry -- Periodicals; Neurochemistry -- Periodicals; Neurochimie -- Périodiques; Neurochemistry; Periodicals; 612.804205
• DOI: 10.1016/j.neuint.2017.08.004
• Languages: English
• ISSNs: 0197-0186

Abstract

Abstract: Neurodegenerative diseases, such as Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD), and Amyotrophic Lateral Sclerosis (ALS), are a complex "family" of pathologies, characterised by the progressive loss of neurons and/or neuronal functions, leading to severe physical and cognitive inabilities in affected patients. These syndromes, despite differences in the causative events, the onset, and the progression of the disease, share as common features the presence of aggregate-prone neuro-toxic proteins, in the form of aggresomes and/or inclusion bodies, perturbing cellular homeostasis and neuronal function (Popovic et al., 2014 ), and the presence of dysfunctional mitochondria. The removal of protein aggregates and of damaged organelles, through the ubiquitin-proteasome system (UPS) and/or the autophagy/lysosome machinery, is a crucial step for the maintenance of neuronal homeostasis. Indeed, their impairment has been reported as associated with the development of these diseases. In this review, we focus on the role played by mitophagy, a specialised form of autophagy, in the onset and progression of major neurodegenerative diseases, as well as on possible therapeutic approaches involving mitophagy modulation. Highlights: The Ubiquitin-proteasome-dependent protein degradation and mitochondrial dynamics are crucial in in neurodegeneration. Macro- and selective-autophagy play distinct roles in neurodegenerative diseases. Mitophagy is crucial in Parkinson's, Alzheimer's, Huntington's diseases and Amyotrophic Lateral Sclerosis. Mitophagy modulation has therapeutic potential in those contextes.