AI Article Synopsis
- Ataxin-3 (AT3) leads to spinocerebellar ataxia type 3 when it has a polyglutamine stretch that exceeds a specific length.
- Research shows that normal AT3 (AT3Q24) and expanded AT3 (AT3Q55) can form oligomers and protofibrils at 37 °C, but only AT3Q55 results in irreversible aggregated fibrils linked by side-chain glutamine hydrogen bonds.
- New findings indicate that gradually heating normal AT3 (to 85 °C) causes similar aggregation as at 37 °C, while AT3Q55 produces large, amorphous aggregates, pointing to how temperature influences fibrillogenesis outcomes.
Article Abstract
Ataxin-3 (AT3) triggers spinocerebellar ataxia type 3 when it carries a polyglutamine stretch expanded beyond a critical threshold. By Fourier transform infrared spectroscopy and atomic force microscopy we previously showed that a normal (AT3Q24) and an expanded (AT3Q55) variant were capable of evolving into oligomers and protofibrils at 37 °C, whereas only the expanded form generated irreversibly aggregated fibrils that also were associated with a network of side-chain glutamine hydrogen bonding [Natalello et al. (2011) PLoS One. 6:e18789]. We report here that AT3Q24, when gradually heated up to 85 °C, undergoes aggregation similar to that observed at 37 °C; in contrast, AT3Q55 only generates large, amorphous aggregates. We propose a possible interpretation of the mechanism by which temperature affects the outcome of fibrillogenesis.
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| http://dx.doi.org/10.1016/j.biochi.2012.01.002 | DOI Listing |
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