In human ALS post-mortem motoneurons, miR-218-2 expression is significantly downregulated and, in a genetic screening of a large ALS cohort, the presence of 6 rare variants in miR-218-2 was functionally associated with its defective processing by DICER (Reichenstein et al., 2019). This evidence concerns the gene DICER1 and amyotrophic lateral sclerosis.