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303,304 Mutations in the SOD1 gene (encoding superoxide dismutase 1) occur in ~20% of cases, 305 while mutations in TARDBP (encoding TAR DNA- binding protein 43 [TDP43]) 306 and FUS (encoding the fused in sarcoma protein) have a lower frequency ( 65) and 10 moderate/severe cases (ACE-R score65), indicating their level of cognitive impairment at the time of their 11 C-PK11195 PET scan