In order to determine if, as previously shown in the hSOD1-G93A mouse model of ALS[14], genetic modifiers of survival are located in the proximal region of chromosome 17, we are developing G59S-hDCTN1 mutant mice on an interval-specific congenic that carries a longer segment of chromosome 17 from NOD mice. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.