This process is usually thought to protect cells against the aggregated protein-induced cell death [13,14,15], and aggresomes formed by pathological proteins have been broadly discovered in neurodegenerative diseases, such as synphilin-1 and DJ-1 aggresomes in Parkinson’s disease, huntingtin aggresomes in Huntington’s disease and copper-zinc superoxide dismutase (SOD1) aggresomes in amyotrophic lateral sclerosis (ALS), respectively [15,16,17,18,19,20,21]. Here, SNCAIP is linked to amyotrophic lateral sclerosis.