Polyglutamine Pathogenesis Emergence of Unifying Mechanisms for Huntington's Disease and Related Disorders

@article{Ross2002PolyglutaminePE,
  title={Polyglutamine Pathogenesis Emergence of Unifying Mechanisms for Huntington's Disease and Related Disorders},
  author={Christopher A. Ross},
  journal={Neuron},
  year={2002},
  volume={35},
  pages={819-822}
}
The mechanisms of neurodegeneration in the CAG repeat polyglutamine diseases, including Spinal and Bulbar Muscular Atrophy (SBMA), Huntington's disease (HD), DentatoRubral and PallidoLuysian Atrophy (DRPLA), and Spino-Cerebellar Ataxia (SCA), have been controversial. Issues have included the role of polyglutamine aggregation and possible amyloid formation, localization in the cell nucleus, and possible proteolytic processing. Proposed mechanisms have included activation of caspases or other… CONTINUE READING