We found that a specific chaperone, the small heat shock protein B8 (HSPB8), recognizes and promotes the removal of the misfolded proteins associated with motoneuron diseases (MNDs) (i.e. mutant SOD1 and a TDP-43 fragment in Amyotrophic Lateral Sclerosis (ALS); androgen receptor with an expanded polyglutamine tract (ARpolyQ) in Spinal and Bulbar Muscular Atrophy (SBMA)) promoting their autophagic removal from motoneurons10, 11, 12, 13, 14. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.