SOD1 and amyotrophic lateral sclerosis: In this regard we have reported that interval-specific congenic mice that carry a chromosome 17 interval (proximal 16 to 53 Mb) derived from SJL on an otherwise pure B6 background (B6.SJL-Chr17) accelerates onset in the transgenic G93A-hSOD1 mouse model of ALS from a B6 phenotype to an SJL phenotype confirming the presence of modifiers in this region.