In conclusion, biochemical and immunohistochemical data obtained by using cell and animal (mouse and Drosophila) models of ALS and other neurodegenerative diseases support the interpretation that HSPB8 has a prominent role in counteracting the toxicity of misfolded proteins in MNDs and may be fundamental in the maintenance of the delicate equilibrium that regulates the routing of proteins to autophagy and to the proteasome. The gene discussed is HSPB8; the disease is neurodegenerative disease.