Article
Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS.
Nature structural biology - 1 Jun 2003
Elam Jennifer Stine, Taylor Alexander B, Strange Richard, Antonyuk Svetlana, Doucette Peter A, Rodriguez Jorge A, Hasnain S Samar, Hayward Lawrence J, Valentine Joan Selverstone, Yeates Todd O, Hart P John
Abstract excerpt
Mutations in the SOD1 gene cause the autosomal dominant, neurodegenerative disorder familial amyotrophic lateral sclerosis (FALS). In spinal cord neurons of human FALS patients and in transgenic mice expressing these mutant proteins, aggregates containing FALS SOD1 are observed. Accumulation of SOD1 aggregates is believed to interfere with axonal transport, protein degradation and anti-apoptotic functions of the...
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