As we showed previously that OXR1 is upregulated in the spinal cord of ALS patients (Oliver et al., 2011), we investigated whether overexpression of OXR1 from early development can delay or prevent motor neuron degeneration by crossing Tg(Prnp-Oxr1) mice with SOD1G93A mice (Gurney et al., 1994). This evidence concerns the gene OXR1 and amyotrophic lateral sclerosis.