[Hereditary chorea--update].

  title={[Hereditary chorea--update].},
  author={Ayako Sano},
  journal={Rinsho shinkeigaku = Clinical neurology},
  volume={44 11},
  • Ayako Sano
  • Published in Rinsho shinkeigaku = Clinical neurology 2004
Understanding molecular genetical background of hereditary chorea has recently been progressed so far. Triplet repeat expansion diseases including, Huntington disease, in which CAG expansion has been identified in the IT-15 or Huntingtin gene, and Huntington disease like-2, in which CTG expansion in junctophilin-3 (JPH3) gene occurs, causes selective degeneration of striatum in the brain. Octapeptide repeat expansion in the prion gene in Huntington disease like-1 has been also identified… CONTINUE READING