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RESEARCH PRODUCT

The Chaperonopathies: Classification, Mechanisms, Structural Features

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The classification of chaperonopathies is presented in this chapter. Like many other diseases, chaperonopathies can be genetic or acquired, primary or secondary, structural and/or functional, and qualitative and/or quantitative. In addition, considering pathogenic mechanism, chaperonopathies can be by defect, excess, or mistake. In the latter, a chaperone is normal but favors disease, a situation that occurs, for instance, in various types of cancers. Structural chaperonopathies are characterized by a change in the molecule of a chaperone due to mutation (genetic chaperonopathy) or due to aberrant post-translational modification (acquired chaperonopathy). In both cases, the impact of the structural change depends on which functional domain within the chaperone molecule is modified.