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Protein Aggregation Diseases: Toxicity of Soluble Prefibrillar Aggregates and Their Clinical Significance

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Protein Misfolding and Cellular Stress in Disease and Aging

Part of the book series: Methods in Molecular Biology ((MIMB,volume 648))

Abstract

Amyloid diseases, the most clinically relevant protein misfolding pathologies due to the high prevalence of some of them in the population, are characterized by the presence, in specific tissues and organs, of fibrillar deposits of specific peptides or proteins. Increasing efforts are presently dedicated at investigating the structural features and the structure-toxicity relation of the soluble oligomeric precursors arising in the path of fibril formation. In fact, it is increasingly recognised that these unstable, dynamic assemblies are remarkably toxic to cells thus featuring these as the main factor responsible for cell impairment in amyloid diseases. This chapter will review shortly the data presently available on the structural and biochemical features of these assemblies, as well as on their biological and clinical significance.

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Acknowledgements

The author gratefully acknowledges financial support from the Ente Cassa di Risparmio di Firenze and Italian MURST (PRIN Project 2007XY59ZJ_001).

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  1. Department of Biochemical Sciences and Research Centre on the Molecular Basis of Neurodegeneration, University of Florence, Florence, Italy

    Massimo Stefani

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  1. Massimo Stefani
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  1. Aarhus University Hospital, Research Unit for Molecular Medicine, University of Aarhus, Brendstrupgaardsvej 100, Aarhus N, 8200, Denmark

    Peter Bross

  2. Aarhus University Hospital, Research Unit for Molecular Medicine, University of Aarhus, Brendstrupgaardsvej 100, Aarhus N, 8200, Denmark

    Niels Gregersen

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Stefani, M. (2010). Protein Aggregation Diseases: Toxicity of Soluble Prefibrillar Aggregates and Their Clinical Significance. In: Bross, P., Gregersen, N. (eds) Protein Misfolding and Cellular Stress in Disease and Aging. Methods in Molecular Biology, vol 648. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-756-3_2

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  • DOI: https://doi.org/10.1007/978-1-60761-756-3_2

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  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-60761-755-6

  • Online ISBN: 978-1-60761-756-3

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