Article
Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS.
Proceedings of the National Academy of Sciences of the United States of America - 16 May 2017
Ciryam Prajwal, Lambert-Smith Isabella A, Bean Daniel M, Freer Rosie, Cid Fernando, Tartaglia Gian Gaetano, Saunders Darren N, Wilson Mark R, Oliver Stephen G, Morimoto Richard I, Dobson Christopher M, Vendruscolo Michele, Favrin Giorgio, Yerbury Justin J
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a heterogeneous degenerative motor neuron disease linked to numerous genetic mutations in apparently unrelated proteins. These proteins, including SOD1, TDP-43, and FUS, are highly aggregation-prone and form a variety of intracellular inclusion bodies that are characteristic of different neuropathological subtypes of the disease. Contained within these inclusions are a...
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