HTT and Huntington disease: Poly-glutamine (poly-Q) amino-terminal truncations of HTT, generated through aberrant splicing of HTT in HD [35], inappropriately accumulate within the nucleus through altered interactions with the nuclear pore protein translocated promoter region (Tpr) [36], and form ubiquitinated neuronal intranuclear inclusions in human [37] and mouse [38] HD brains.