The changing scene of amyotrophic lateral sclerosis

W Robberecht, T Philips - Nature Reviews Neuroscience, 2013 - nature.com
W Robberecht, T Philips
Nature Reviews Neuroscience, 2013nature.com
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 ribonucleopathy 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, myotonic dystrophy …
Abstract
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 ribonucleopathy 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, myotonic 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.
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