Recent research has provided evidence that nuclear pore complex (NPC) function is disrupted in normal aging and dysfunction may contribute to neuronal loss in several neurodegenerative diseases, including frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), Parkinson’s disease (PD) and HD (Patel and Chu, 2011; Zhang et al., 2015; Grima et al., 2017; Chou et al., 2018). This evidence concerns the gene NPC1 and Huntington disease.