Among the canonical MyomiRs (miR-1, -133a/b and -206), miR-206 was found to be upregulated in the synaptic region of G93A-SOD1 mice and in the skeletal muscle of ALS patients, and seems to promote a partially successful compensatory response to skeletal muscle denervation, a common event during disease progression. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.