Hsp60 Friend and Foe of the Nervous System

Hsp60 belongs to the subgroup of molecular chaperones named chaperonins and, typically, resides and functions in the mitochondria but it is also present inextramitochondrial sites. It chaperones client peptides as they fold to achieve thenative conformation and also displays anti-stress roles by helping stress-damagedproteins regain a functional shape. Thus, Hsp60 is central to the integrity and functionality of mitochondria and energy production. All cells in the nervous systemdepend on Hsp60 so when the chaperonin malfunctions the consequences on nervous tissues are usually devastating, causing diverse diseases. These are the Hsp60chaperonopathies, which can be genetic or acquired with the former caused by genevariants and the latter by various post-transcriptional mechanisms. All forms ofchaperonopathies, i.e., by defect, by excess, and by mistake, associated with Hsp60have been described, and some illustrative examples are discussed here. It is clearthat this chaperonin is key to neuromuscular physiology but, when qualitativelyand/or quantitatively abnormal causes diseases, often very serious.