The gene encodes for a deubiquitinase (DUB), a class of enzymes that counteracts the action of ubiquitin and important for the control protein stability of TP-43 and HTT (Doss-Pepe et al., 2003; van Well et al., 2019; Tran and Lee, 2022), responsible when mutated of ALS and HD, making DUBs potential targets for proteinopathies. This evidence concerns the gene HTT and Huntington disease.