Structural formation of huntingtin exon 1 aggregates probed by small-angle neutron scattering.

ABSTRACT: In several neurodegenerative disorders, including Huntington's disease, aspects concerning the earliest of protein structures that form along the aggregation pathway have increasingly gained attention because these particular species are likely to be neurotoxic. We used time-resolved small-angle neutron scattering to probe in solution these transient structures formed by peptides having the N-terminal sequence context of mutant huntingtin exon 1. We obtained snapshots of the formed aggregates as the kinetic reaction ensued to yield quantitative information on their size and mass. At the early stage, small precursor species with an initial radius of gyration of 16.1 ± 5.9 Å and average mass of a dimer to trimer were monitored. Structural growth was treated as two modes with a transition fro

SUBMITTER: Stanley CB 

PROVIDER: S-EPMC3093554 | biostudies-literature | 2011 May

REPOSITORIES: biostudies-literature