Article
Amyotrophic lateral sclerosis: Problems and prospects.
Annals of neurology - 1 Sept 2013
Sreedharan Jemeen, Brown Robert H
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a lethal degenerative disorder of motoneurons, which may occur concurrently with frontotemporal dementia. Genetic analyses of the ∼10% of ALS cases that are dominantly inherited provide insight into ALS pathobiology. Two broad themes are evident. One, prompted by investigations of the SOD1 gene, is that conformational instability of proteins triggers downstream neurotoxic...
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