To probe the potential mechanisms and domain requirements of the hHSJ1a mediated reduction in SOD1 aggregation and improved motor neuron survival in the SOD1G93A mouse model of ALS, we developed a cell model of SOD1G93A aggregation based on the expression of GFP tagged SOD1 (GFP-SOD1) in SK-N-SH neuroblastoma cells, which do not express detectable endogenous HSJ1. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.