Recently, many reports have shown that these NOTCH2NLC mRNAs containing extended GGC repeats colocalize with p62 in nuclear inclusion bodies of patients with NIID, as well as in many RNA-binding proteins (Sam68, hnRNPA2/B1, MBNL1, DGCR, etc.)sequestered in the secondary structure of NOTCH2NLC mRNAs (Glineburg et al., 2018; Deng et al., 2021). Here, KHDRBS1 is linked to neuronal intranuclear inclusion disease.