Transcriptional dysregulation in Huntington's disease.
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.
Cha
J H
JH
Dept. of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
D006801:Humans; D006816:Huntington Disease / Q000235:genetics* / Q000503:physiopathology*; D014158:Transcription, Genetic / Q000502:physiology*