PROTEOSTASIS AS A SIGNALING DEVICE AND THERAPEUTIC TARGET

The term ‘conformational disorders’ was created recently for the numerous pathological situations due to the accumulation of aberrant proteins (or normal ones in excess). However, defective protein homeostasis (or proteostasis) is not always associatedwith pathology, as it controls the life-span of certain cells and other ‘physiological aging’ signaling processes (in quotes becausehumans are generally reluctant to consider aging a physiological process). As proteotoxicity emerged as the underlying cause of many degenerative diseases, mechanisms controlling proteostasis become crucial pharmacological targets. This chapter summarizes the emerging concepts and some recent results that allow some optimism in the treatment of different severe diseases via the manipulation of proteostasis.