However, it has been shown in HD models, that also key components of the Ran‐mediated transport system, including Ran itself and RanGAP, are affected and downregulated in the nucleus, possibly leading to mis‐localization of mHtt and other pathogenic proteins (Liu et al, 2015; Gasset‐Rosa et al, 2017; Grima et al, 2017). This evidence concerns the gene RANGAP1 and Huntington disease.