Article
Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase.
Neurobiology of aging - 1 Dec 2012
Farg Manal A, Soo Kai Y, Walker Adam K, Pham Hong, Orian Jacqueline, Horne Malcolm K, Warraich Sadaf T, Williams Kelly L, Blair Ian P, Atkin Julie D
Abstract excerpt
Mutations in the gene encoding fused in sarcoma (FUS) are linked to amyotrophic lateral sclerosis (ALS), but the mechanisms by which these mutants trigger neurodegeneration remain unknown. Endoplasmic reticulum (ER) stress is increasingly recognized as an important and early pathway to motor neuron death in ALS. FUS is normally located in the nucleus but in ALS, FUS redistributes to the cytoplasm and forms...
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