The Chaperonopathies - Diseases with Defective Molecular Chaperones

Abstract

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 characteristic. Overview and Book Plan -- Chaperones: General Characteristics and Classifications -- The Chaperonopathies: Classification, Mechanisms, Structural Features -- Structural and Hereditary Chaperonopathies: Mutation -- Other Genetic Chaperonopathies -- Acquired Chaperonopathies -- Other Types of Chaperonopathies -- Impact of Chaperonopathies in Protein Homeostasis and Beyond -- Extracellular Chaperones.