Article
Intermolecular transmission of superoxide dismutase 1 misfolding in living cells.
Proceedings of the National Academy of Sciences of the United States of America - 27 Sept 2011
Grad Leslie I, Guest Will C, Yanai Anat, Pokrishevsky Edward, O'Neill Megan A, Gibbs Ebrima, Semenchenko Valentyna, Yousefi Masoud, Wishart David S, Plotkin Steven S, Cashman Neil R
Abstract excerpt
Human wild-type superoxide dismutase-1 (wtSOD1) is known to coaggregate with mutant SOD1 in familial amyotrophic lateral sclerosis (FALS), in double transgenic models of FALS, and in cell culture systems, but the structural determinants of this process are unclear. Here we molecularly dissect the effects of intracellular and cell-free obligately misfolded SOD1 mutant proteins on natively structured wild-type...
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