In European populations, the most common mutations in ALS were the C9orf72 repeats, followed by SOD1, ATXN2 repeats, FUS, TARDBP, VCP, UBQLN2 and SQSTM1, In Asian populations, the most common mutations in ALS were the SOD1, followed by C9orf72 repeats, FUS, ATXN2 repeats, SQSTM1, TARDBP, UBQLN2, and VCP. Our results suggested that gene mutations are more common in European than Asians, and the mutant spectrum in different populations is significant differences. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.