The changing scene of amyotrophic lateral sclerosis

被引:751
|
作者
Robberecht, Wim [1 ,2 ,3 ,4 ]
Philips, Thomas [1 ,2 ,3 ,5 ]
机构
[1] VIB Vesalius Res Ctr, Neurobiol Lab, B-3000 Louvain, Belgium
[2] Univ Leuven KU Leuven, Expt Neurol Dept Neurosci, B-3000 Louvain, Belgium
[3] Univ Leuven KU Leuven, Leuven Res Inst Neurosci & Dis LIND, B-3000 Louvain, Belgium
[4] Katholieke Univ Leuven Hosp, Dept Neurol, B-3000 Louvain, Belgium
[5] Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21205 USA
关键词
SPINAL MUSCULAR-ATROPHY; NUCLEAR FACTOR TDP-43; FRONTOTEMPORAL LOBAR DEGENERATION; HEXANUCLEOTIDE REPEAT EXPANSION; LENGTH POLYGLUTAMINE EXPANSIONS; TRANSGENIC MOUSE MODEL; RNA-BINDING PROTEINS; CORD MOTOR-NEURONS; PRE-MESSENGER-RNA; MUTANT SOD1;
D O I
10.1038/nrn3430
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Several recent breakthroughs have provided notable insights into the pathogenesis of amyotrophic lateral sclerosis (ALS), with some even shifting our thinking about this neurodegenerative disease and raising the question as to whether this disorder is a proteinopathy, a ribonucteopathy or both. In addition, these breakthroughs have revealed mechanistic links between ALS and frontotemporal dementia, as well as between ALS and other neurodegenerative diseases, such as the cerebellar atrophies, nnyotonic dystrophy and inclusion body myositis. Here, we summarize the new findings in ALS research, discuss what they have taught us about this disease and examine issues that are still outstanding.
引用
收藏
页码:248 / 264
页数:17
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