While X chromosome inactivation reduces the impact of FXTAS in females, female premutation carriers face an increased risk of developing Fragile X-Associated Primary Ovarian Insufficiency (FXPOI) and of passing on a full FMR1 mutation, which can lead to Fragile X Syndrome (FXS) in their offspring [1,2,3,4,5]. The gene discussed is FMR1; the disease is fragile X syndrome.