The Neurochaperonopathies: Anomalies of the Chaperone System with Pathogenic Effects in Neurodegenerative and Neuromuscular Disorders

The chaperone (or chaperoning) system (CS) constitutes molecular chaperones, co-chaperones, and co-factors, interactors receptors, its canonical role is protein quality control. A malfunction of the CS may cause diseases, known as chaperonopathies. These are caused by qualitatively and/or quantitatively abnormal chaperones. Since ubiquitous, chaperonopathies systemic, affecting various tissues organs, playing an etiologic-pathogenic in diverse conditions. In this review, we focus on involved pathogenic mechanisms diseases central peripheral nervous systems: neurochaperonopathies (NCPs). Genetic NCPs linked to variants genes encoding, for example, small Hsp, Hsp10, Hsp40, Hsp60, CCT-BBS (chaperonin-containing TCP-1- Bardet–Biedl syndrome) Instead, acquired associated with malfunctional such Hsp70, Hsp90, VCP/p97 aberrant post-translational modifications. Awareness underlying primary or secondary causes disease will improve diagnosis patient management open possibility investigating developing chaperonotherapy, namely treatment main target. Positive chaperonotherapy would apply defect, i.e., insufficiency, consist replacement boosting, whereas negative be pertinent when a actively participates initiation progression must blocked eliminated.