The pathology and alterations in expression of RNA binding proteins such as mutant SOD1 (mtSOD1), TDP-43 and FUS/TLS in ALS, as well as evidence of mutations in these proteins being associated with altered RNA processing, has suggested that alterations in the metabolism of RNA may be critical to the pathogenesis of ALS [1,6,8,11]. Here, SOD1 is linked to amyotrophic lateral sclerosis.