Since an abnormal disappearance of TDP-43 from nuclei was observed in motor neurons from sporadic ALS patients and Gems were lost in these neurons (Fig 3) and TDP-43 was important for maintaining the proper integrity of spliceosome U snRNPs (Fig 4), we thought it would be of high interest to investigate whether U snRNA and U snRNP misregulation occurs in affected regions of ALS patients. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.