Article
Detection of Mutant Huntingtin Aggregation Conformers and Modulation of SDS-Soluble Fibrillar Oligomers by Small Molecules.
Journal of Huntington's disease - 1 Jan 2012
Sontag Emily Mitchell, Lotz Gregor P, Yang Guocheng, Sontag Christopher J, Cummings Brian J, Glabe Charles G, Muchowski Paul J, Thompson Leslie Michels
Abstract excerpt
The Huntington's disease (HD) mutation leads to a complex process of Huntingtin (Htt) aggregation into multimeric species that eventually form visible inclusions in cytoplasm, nuclei and neuronal processes. One hypothesis is that smaller, soluble forms of amyloid proteins confer toxic effects and contribute to early cell dysfunction. However, analysis of mutant Htt aggregation intermediates to identify conformers...
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