Nucleocytoplasmic transport of huntingtin and Huntington's disease

One historic characteristic of Huntington's disease (HD), which affected brains in both patient studies and HD mouse models was the detection of elevated levels of huntingtin protein fragments (NNI, neuronal nuclear inclusions) in the nuclei of several neuronal sub-types. This nuclear localization of mutant huntingtin fragments has been implicated as a potential gain of function for polyglutamine expanded huntingtin and may indicate the …