In conclusion, our study is the first characterization of a panel of 8 lncRNAs (linc-Enc1, linc–Brn1a, linc–Brn1b, linc-p21, Hottip, Tug1, Eldrr, and Fendrr) in ALS, indicating a deregulation in all of their expression in specific ALS affected areas or at different stages of the pathogenesis. Here, HOTTIP is linked to amyotrophic lateral sclerosis.