SOD1 and amyotrophic lateral sclerosis: Members of the Hsp70 family and their J-domain partner protein and sHsp chaperones have been found to colocalize with SOD1 inclusions in patient tissues and rodent ALS models and to interact with mutant SOD1 in cell culture models, indicating that aggregated SOD1 is recognized as a substrate by the PQC system (Shinder et al., 2001; Watanabe et al., 2001; Howland et al., 2002; Liu et al., 2005; Matsumoto et al., 2005).