This mechanism might resolves a long-standing paradox that on the one hand, many mature ALS-causing mutants such as G93A-hSOD1 have their mature structures and activity almost identical to those of the mature WT hSOD147, but on the other hand, in vivo they have been found to be highly toxic and aggregation-prone31, 68, 72. Here, SOD1 is linked to amyotrophic lateral sclerosis.