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Mutations in Ubiquilin2 (UBQLN2) gene have been identified in familial forms of Amyotrophic lateral sclerosis (fALS) and ALS/dementia. Ubiquilin2 pathology was also described in both non-UBQLN2 fALS and in sporadic ALS cases. Ubiquilin2 is involved in degradation of ubiquitinated proteins, and functional studies have shown the formation of abnormal protein aggregation in Ubiquilin2 overexpressing...