Alteration of protein folding and degradation in motor neuron diseases: Implications and protective functions of small heat shock proteins

► Several motor neuron diseases are associated to aberrant protein folding (misfolding). ► Protein misfolding results in aggregation as well as in the perturbation of several intracellular functions. ► Molecular chaperones, the proteasome and the autophagy are responsible for the clearance of misfolded proteins. ► Three members of the HSPB family have been found mutated in different motor neuron diseases. ► A member of the small heat shock protein (HSPB) family, HSPB8 facilitates the autophagic removal of misfolded proteins exerting protective role on motor neurons.