Huntington disease (HD) is an adult-onset neurodegenerative disease caused by expansion
of a polyglutamine (poly (Q) tract in the N-terminal region of huntingtin (htt). Although the
precise mechanisms leading to neurodegeneration in HD have not been fully elucidated,
transcriptional dysregulation has been implicated in disease pathogenesis. In HD, multiple
N-terminal mutant htt fragments smaller than the first 500 amino acids have been found to
accumulate in the nucleus and adversely affect gene transcription. It is unknown whether …