Interestingly, expression of mutant forms and not WT, of ubiquilin-2 also resulted in inclusions or aggregates that stained positive for ThioflavinS suggesting induction of amyloid pathology (Fig. 7) further supporting the notion that ALS-linked ubiquilin-2 mutants induce proteinopathy via misfolding and aggregation of proteins. The gene discussed is UBQLN2; the disease is amyotrophic lateral sclerosis.