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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. "
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