Several families of proteins called molecular chaperones comprise the cellular machinery that has evolved to maintain protein structure and eliminate misfolded proteins in the cell. In experimental animal models, chaperones have been shown to be powerful inhibitors of neurodegeneration. As such, molecular chaperones represent exciting pharmaceutical targets that potentially eliminate aberrant cellular proteins and slow disease progression. Current evidence indicates that the J protein family is the basis of selective chaperone action in the cell. Hence, J proteins are currently attracting attention as novel therapeutic targets for a number of neurodegenerative disorders.