Transcriptional dysregulation in Huntington's disease

Trends Neurosci. 2000 Sep;23(9):387-92. doi: 10.1016/s0166-2236(00)01609-x.


Although the gene responsible for Huntington's disease was discovered in 1993, the pathogenic mechanisms by which mutant huntingtin causes neuronal dysfunction and death remain unclear. However, increasing evidence suggests that mutant huntingtin disrupts the normal transcriptional program of susceptible neurons. Thus, transcriptional dysregulation might be an important pathogenic mechanism in Huntington's disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Humans
  • Huntington Disease / genetics*
  • Huntington Disease / physiopathology*
  • Transcription, Genetic / physiology*