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Dexpramipexole Dexpramipexole, an enantiomer of pramipexole, is believed to increase ATP generation by improving efficiency of oxidative phosphorylation as well as reducing oxidative damage of mitochondria (Gribkoff and Bozik, In vitro studies showed that it is able to promote metabolic efficiency of injured cells and scavenging of free radicals in the mitochondria (Danzeisen et al., 2 O 2 mediated cell death and prevented the generation of mitochondrial reactive oxygen species in neuroblastoma cells (Ferrari-Toninelli et al., G93A mouse model, dexpramipexole preserved motor function and prolonged life span (Danzeisen et al., in vitro and in vivo , dexpramipexole was investigated in a double blind randomized phase 2 clinical trial in patients with ALS, showing that treatment for 12 months was safe and well tolerated (Cudkowicz et al., 2013), in rat primary cortical neurons transfected with human mutant TDP-43 as well as in the SOD1 G93A mouse model (Vieira et al., 2014) showed no beneficial effect