Article
Atomic structure of a toxic, oligomeric segment of SOD1 linked to amyotrophic lateral sclerosis (ALS).
Proceedings of the National Academy of Sciences of the United States of America - 15 Aug 2017
Sangwan Smriti, Zhao Anni, Adams Katrina L, Jayson Christina K, Sawaya Michael R, Guenther Elizabeth L, Pan Albert C, Ngo Jennifer, Moore Destaye M, Soriaga Angela B, Do Thanh D, Goldschmidt Lukasz, Nelson Rebecca, Bowers Michael T, Koehler Carla M, Shaw David E, Novitch Bennett G, Eisenberg David S
Abstract excerpt
Fibrils and oligomers are the aggregated protein agents of neuronal dysfunction in ALS diseases. Whereas we now know much about fibril architecture, atomic structures of disease-related oligomers have eluded determination. Here, we determine the corkscrew-like structure of a cytotoxic segment of superoxide dismutase 1 (SOD1) in its oligomeric state. Mutations that prevent formation of this structure eliminate...
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