SOD1 and amyotrophic lateral sclerosis: Moreover, the presence of Cu-deficient SOD1 in the disease-affected spinal cords of ALS model mice has been confirmed; direct assessment of metals bound to SOD1 via a quantitative mass spectrometry approach38 shows that almost half of the total SOD1 pool in the spinal cords of SOD1G37R mice is Cu-deficient and a similar pool of Cu-deficient SOD1 is present in the spinal cords of SOD1G93A mice10.