Similar to these neurodegenerative diseases, the impairment of the UPS, such as the increase of ubiquitinated proteins and P62 protein, is found in the pathological changes of NIID (Oh et al., 2015; Hagerman and Hagerman, 2021; Boivin and Charlet-Berguerand, 2022; Ogasawara et al., 2022; Ribot et al., 2022; Zhou et al., 2022). Here, KHDRBS1 is linked to neuronal intranuclear inclusion disease.