Individuals with repeat numbers between 55 and 200 are referred to as premutation carriers and may develop other FMR1-associated disorders, such as fragile-X-associated tremor ataxia syndrome (FXTAS) for males and some females and fragile-X-associated primary ovarian insufficiency (FXPOI) for females [1,3,16,17,18]. The gene discussed is PSMB5; the disease is fragile X-associated tremor/ataxia syndrome.