Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease.

@article{Weiss2008SensitiveBA,
  title={Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease.},
  author={Andreas M. Weiss and Corinna Klein and Ben Woodman and Kirupa Sathasivam and Miriam Bibel and Etienne R{\'e}gulier and Gillian P Bates and Paolo Paganetti},
  journal={Journal of neurochemistry},
  year={2008},
  volume={104 3},
  pages={846-58}
}
A CAG-repeat gene expansion translated into a pathogenic polyglutamine stretch at the N-terminus of huntingtin triggers Huntington's Disease. Mutated huntingtin is predicted to adopt toxic properties mainly if aggregation-prone N-terminal fragments are released by proteolysis. Huntingtin-aggregates are indeed a major hallmark of this disorder and could… CONTINUE READING