UBQLN2 and amyotrophic lateral sclerosis: On the X chromosome we identified a female centenarian with a previously described mutation in UBQLN2 P525S (Deng et al. 2011), which was found to cause an X-linked dominant type of familial amyotrophic lateral sclerosis (ALS) and ALS/dementia with an estimated penetrance of approximately 90% (Table 1).