Mitophagy in neurodegenerative diseases

被引:73
作者
Rodolfo, Carlo [1 ]
Campello, Silvia [1 ,2 ]
Cecconi, Francesco [1 ,3 ]
机构
[1] Univ Roma Tor Vergata, Dept Biol, I-00133 Rome, Italy
[2] IRCCS Fdn Santa Lucia, I-00143 Rome, Italy
[3] IRCCS Bambino Gesu Childrens Hosp, Dept Pediat Haematol & Oncol, I-00146 Rome, Italy
关键词
ABNORMAL MITOCHONDRIAL DYNAMICS; AMYOTROPHIC-LATERAL-SCLEROSIS; DEFECTIVE AXONAL-TRANSPORT; PARKIN-MEDIATED MITOPHAGY; MACHADO-JOSEPH-DISEASE; PROTEIN B8 HSPB8; MOUSE MODEL; MUTANT HUNTINGTIN; AMYLOID-BETA; SYNAPTIC DEGENERATION;
D O I
10.1016/j.neuint.2017.08.004
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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. (C) 2017 Elsevier Ltd. All rights reserved.
引用
收藏
页码:156 / 166
页数:11
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