Although no association was apparent between SFPQ nuclear signal intensity and presence or absence of HD-associated intranuclear inclusions in striatal and cortical neurons of seven HD cases (Baskota et al., 2019), the reduced nuclear availability of free FUS in HD and, as a consequence, a decreased interaction with SFPQ, might affect 4R-Tau/3R-Tau ratio. Here, SFPQ is linked to Huntington disease.