Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization.

  title={Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization.},
  author={Michelle A. Poirier and Huilin Li and Jed C. Macosko and Shuowei Cai and Mario L Amzel and Christopher A. Ross},
  journal={The Journal of biological chemistry},
  volume={277 43},
The pathology of Huntington's disease is characterized by neuronal degeneration and inclusions containing N-terminal fragments of mutant huntingtin (htt). To study htt aggregation, we examined purified htt fragments in vitro, finding globular and protofibrillar intermediates participating in the genesis of mature fibrils. These intermediates were high in beta-structure. Furthermore, Congo Red, a dye that stains amyloid fibrils, prevented the assembly of mutant htt into mature fibrils, but not… CONTINUE READING
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