SOD1 and amyotrophic lateral sclerosis: These studies were conducted using SOD1-G93A transgenic mice (strain B6.Cg-Tg (SOD1*G93A)1Gur/J), which express an ALS-linked human mutant allele of SOD1 [26] and are the in vivo model of choice for preclinical efficacy studies in ALS [27, 28].