This Brief provides a concise review of chaperonopathies, i.e., diseases
in which molecular chaperones play an etiologic-pathogenic role.
Introductory chapters deal with the chaperoning system and chaperoning
teams and networks, HSP-chaperone subpopulations, the locations and
functions of chaperones, and chaperone genes in humans. Other chapters
present the chaperonopathies in general, including their molecular
features and mechanistic classification into by defect, excess, or
mistake. Subsequent chapters discuss the chaperonopathies in more
detail, focusing on their distinctive characteristics: primary or
secondary; quantitative and/or qualitative; structural and hereditary or
acquired; genetic polymorphisms; gene dysregulation; age-related;
associated with cancer, chronic inflammatory conditions, and autoimmune
diseases. The interconnections between the chaperoning and the immune
systems in cancer development, chronic inflammation, autoimmunity, and
ageing are outlined, which leads to a discussion on the future prospects
of chaperonotherapy. The latter may consist of chaperone gene and
protein replacement/supplementation in cases of deficiency and of gene
or protein blocking when the chaperone actively promotes disease. The
last chapter presents the extracellular chaperones and details on how
the chaperone Hsp60 is secreted into the extracellular space and, thus,
appears in the blood of cancer patients with potential to participate in
carcinogenesis and chronic inflammation and autoimmunity. Chaperones as
clinically useful biomarkers are mentioned when pertinent. Likewise,
guidelines for clinical evaluation of chaperonopathies and for their
histopathological and molecular identification are provided throughout.
The book also provides extensive bibliography organized by chapter and
topic with comments.
