The striking up-regulation of intermediate OXR1 isoforms we observed in ALS may be a consequence of significant neurodegeneration in the spinal cord; but crucially, full-length Oxr1 protein levels were significantly increased in SOD1 G93A mice before any overt phenotypic abnormalities, suggesting Oxr1 may be an early marker of neurodegeneration [30]–[31], [38]–[39]. Here, OXR1 is linked to amyotrophic lateral sclerosis.