Abstract
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.
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Further Reading See also Chap. 2 for Further Reading
Sections 3.1–3.3 Chaperonopathies vs. Proteinopathies
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PROTEINOPATHIES Quantitative Changes in Chaperones and Chaperones in Pathological Protein Precipitates
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Proteinopathies and Proteotoxicity
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Filaminopathies
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Tauopathies, for instance Alzheimer’s disease
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Alpha-Synucleinopathies: For Instance Parkinson’s Disease
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Polyglutamine Expansion Diseases: For Instance Huntington’s Disease and Some Ataxias
Kubota H, Kitamura A, Nagata K (2011) Analyzing the aggregation of polyglutamine-expansion proteins and its modulation by molecular chaperones. Methods 53:267–274
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Prion Diseases
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PATHOLOGICAL “AMATEUR” CHAPERONES
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POST-TRANSLATIONAL MODIFICATIONS OF CHAPERONES AND IMPACT ON FUNCTION
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Macario, A.J., Conway de Macario, E., Cappello, F. (2013). The Chaperonopathies: Classification, Mechanisms, Structural Features. In: The Chaperonopathies. SpringerBriefs in Biochemistry and Molecular Biology. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-4667-1_3
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DOI: https://doi.org/10.1007/978-94-007-4667-1_3
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