Chapter 12 - Chaperone Proteins and Chaperonopathies

Add time:09/01/2019    Source:sciencedirect.com

Chaperonopathies are caused by qualitatively and/or quantitatively abnormal molecular chaperones. These are proteins, chaperoning system components along with co-chaperones, chaperone co-factors, receptors, and interactors. Any of them can be etiopathogenic in chaperonopathies. Chaperones can be structurally/functionally deficient because of a genetic alteration (e.g., mutation), usually hereditary, or because of a nonhereditary modification (e.g., aberrant posttranslational modification). Mechanistically, chaperonopathies can be by defect (quantitatively or qualitatively), excess (quantitatively or qualitatively), or mistake. In the latter, an apparently normal (as far as current methodology can determine) chaperone promotes disease. This happens in cancers that need chaperones to grow and metastasize and in chronic inflammatory and autoimmune conditions (the pathogenic chaperone stimulates inflammation or is autoantigenic). Chaperonopathies can affect any tissue and therefore are of interest across many medical specialties. Diagnosis and patient monitoring focus on chaperones, which are disease biomarkers. Treatment also targets chaperones (chaperonotherapy) to block or replace/boost them as required. This is important, considering also the key roles of chaperones in the antistress mechanisms.

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