An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils.

@article{Bevivino2001AnEG,
  title={An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils.},
  author={A E Bevivino and Patrick J. Loll},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2001},
  volume={98 21},
  pages={11955-60}
}
The protein ataxin-3 contains a polyglutamine region; increasing the number of glutamines beyond 55 in this region gives rise to the neurodegenerative disease spinocerebellar ataxia type 3. This disease and other polyglutamine expansion diseases are characterized by large intranuclear protein aggregates (nuclear inclusions). By using full-length human ataxin-3, we have investigated the changes in secondary structure, aggregation behavior, and fibril formation associated with an increase from… CONTINUE READING

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