Finally, similar to our observations, impaired miRNA biogenesis at Dicer level associated with downregulation of miRNA levels and reorganization of Dicer and Ago-2 complexes has recently been described in amyotrophic lateral sclerosis [73] suggesting that miRNA malfunction could be a contributor to pathogenesis associated with protein-misfolding associated diseases. This evidence concerns the gene DICER1 and amyotrophic lateral sclerosis.