ATXN10 and myotonic dystrophy type 1: In contrast, diseasessuch as myotonic dystrophy type 1 (DM1) (54,55), fragile X-associatedtremor ataxia syndrome (FXTAS), myotonic dystrophy type 2 (DM2), SCA31, SCA10,SCA8, and, more recently, amyotrophic lateral sclerosis and frontotemporalsclerosis have been associated with an RNA gain-of-function mechanism in whichthe trinucleotide expansion leads to the formation of nuclear RNA foci thatsequester specific RNA-binding proteins (5,56,57).