These four mutations have been previously shown to abrogate the protective activity of DJ-1 (Chen et al., 2010, Shendelman et al., 2004, Taira et al., 2004), with one in particular (homozygous mutation in exon 7, E163) having been identified in an Italian family with three brothers affected by early-onset parkinsonism, dementia, and amyotrophic lateral sclerosis (Annesi et al., 2005). This evidence concerns the gene PARK7 and amyotrophic lateral sclerosis.