CCS and amyotrophic lateral sclerosis: This copper complex readily crosses the BBB with high membrane-permeability [107,108] and is supposed to facilitate the CCS-dependent activation of mutant SOD1 within the cell; in that sense, it will be interesting to test if the disease symptoms of ALS model mice can be ameliorated by the supplementation of CuII(atsm) even in the absence of CCS gene.