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  • © 2013

The Chaperonopathies

Diseases with Defective Molecular Chaperones

  • A unique and comprehensive survey and description of diseases, the chaperonopathies, in which molecular chaperones are etiologic-pathogenic factors
  • An easy-to-grasp, well-illustrated with tables and figures, panoramic view of the state-of-the-art analysis of chaperonopathies
  • Unveils new routes to diagnose chaperonopathies, establish prognosis, treat and manage patients properly, thereby showing the path for future research

Part of the book series: SpringerBriefs in Biochemistry and Molecular Biology (BRIEFSBIOCHEM)

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Table of contents (9 chapters)

  1. Front Matter

    Pages i-xvi
  2. Overview and Book Plan

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 1-14
  3. Chaperones: General Characteristics and Classifications

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 15-33
  4. The Chaperonopathies: Classification, Mechanisms, Structural Features

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 35-42
  5. Structural and Hereditary Chaperonopathies: Mutation

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 43-62
  6. Other Genetic Chaperonopathies

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 63-69
  7. Acquired Chaperonopathies

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 71-74
  8. Other Types of Chaperonopathies

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 75-106
  9. Impact of Chaperonopathies in Protein Homeostasis and Beyond

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 107-108
  10. Extracellular Chaperones

    • Alberto J.L Macario, Everly Conway de Macario, Francesco Cappello
    Pages 109-116

About this book

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. 

Authors and Affiliations

  • School of Medicine, Dept. of Microbiology and Immunology, University of Maryland, Baltimore, USA

    Alberto J.L. Macario

  • Biotechnology Inst., Department of Microbiology and Immunolog, University of Maryland, Baltimore, USA

    Everly Conway de Macario

  • , Department of Experimental Biomedicine, University of Palermo, Palermo, Italy

    Francesco Cappello

Bibliographic Information

Buy it now

Buying options

eBook USD 39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Other ways to access