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If administered to hSOD1 G93A /hCCS mice during the presymptomatic disease phase Cu II (atsm) prevented the development of ALS altogether, [154] whilst administration to hSOD1 G93A and hSOD1 G37R mice after symptom onset markedly improved locomotor function and survival time.[ 228 , 230 ] Importantly, the therapeutic benefit of this compound across numerous mutant SOD1 transgenic mouse strains has been independently verified by multiple research groups.[ 154 , 231 ] Accordingly, phase II/III clinical trials of Cu II (atsm) have recently begun in a small cohort of ALS patients ( n= 80

doi: 10.3390/ANTIOX12091674 272 NanceSADuncanAVGwathmeyTMHairstonKG

10.3390/ijms22137229 46 LudinA.Gur-CohenS.GolanK.KaufmannK