“…Transgenic C. elegans expressing the human G93A SOD1 variant, associated with familial amyotrophic lateral sclerosis (ALS), in motor neurons show motor defects and increased autophagy in an age-dependent manner (Aksenov et al, 2013; Li et al, 2013). SOD-1 overexpression has also been associated with mitochondrial swelling, and learning and memory impairment in flies, mice, and humans (Shin et al, 2004; Perluigi and Butterfield, 2012; Haddadi et al, 2014). For example, transgenic flies expressing a zinc-deficient SOD1 mutant display behavioral defects, including impairment of locomotion, associated with mitochondrial respiratory chain deficiency and matrix vacuolization, that is not accompanied by shortening of lifespan (Bahadorani et al, 2013).…”