Pathways to mitochondrial dysfunction in ALS pathogenesis

被引:76
作者
Carri, Maria Teresa [1 ,2 ]
D'Ambrosi, Nadia [3 ]
Cozzolino, Mauro [4 ]
机构
[1] Univ Roma Tor Vergata, Dept Biol, Via Ric Sci, I-00133 Rome, Italy
[2] Fdn Santa Lucia, IRCCS, Via Fosso Fiorano 64, I-00143 Rome, Italy
[3] Univ Cattolica Sacro Cuore, Inst Anat & Cell Biol, Largo Francesco Vito 1, I-00168 Rome, Italy
[4] CNR, Inst Translat Pharmacol, Via Fosso Cavaliere 100, I-00133 Rome, Italy
关键词
Amyotrophic Lateral Sclerosis; Mitochondria; Protein aggregation; Mitophagy; Alternative splicing; AMYOTROPHIC-LATERAL-SCLEROSIS; PRION-LIKE PROPAGATION; MUTANT SOD1; FRONTOTEMPORAL DEMENTIA; CALCIUM HOMEOSTASIS; AUTOPHAGY RECEPTOR; AXONAL-TRANSPORT; C9ORF72; DYNAMICS; AGGREGATION;
D O I
10.1016/j.bbrc.2016.07.055
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
070307 [化学生物学]; 071010 [生物化学与分子生物学];
摘要
Alterations in the structure and functions of mitochondria are a typical trait of Amyotrophic Lateral Sclerosis, a neurodegenerative disease characterized by a prominent degeneration of upper and lower motor neurons. The known gene mutations that are responsible for a small fraction of ALS cases point to a complex interplay between different mechanisms in the disease pathogenesis. Here we will briefly overview the genetic and mechanistic evidence that make dysfunction of mitochondria a candidate major player in this process. (C) 2016 Elsevier Inc. All rights reserved.
引用
收藏
页码:1187 / 1193
页数:7
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