Examination of protein inclusions in pathological tissue from both sporadic ALS and ALS-dementia demonstrate the presence of ubiquilin-2 in inclusions and co-localization with other proteins such as ubiquitin and p62/SQSTM1, further suggesting a role for ubiquilin-2 in proteinopathy and in ALS pathology [1, 8, 9]. This evidence concerns the gene SQSTM1 and amyotrophic lateral sclerosis.