IMR Press / FBL / Volume 15 / Issue 1 / 10.2741/3626

Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.

Open Access Article
Methods and models in neurodegenerative and systemic protein aggregation diseases
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1 Molecular Biotechnology/IFM, Linkoping University, SE-581 83, Linkoping, Sweden
2 Department of Chemistry, University of Cambridge, Lensfield Road, Cambridge, CB2 1EW, UK
3 Cambridge Institute for Medical Research, Wellcome/MRC Building, Hills Road, Cambridge, CB2 0XY, UK
4 Department of Genetics, University of Cambridge, Downing Street, Cambridge, CB2 3EH, UK
5 Nanoscience Centre, University of Cambridge, J. J. Thomson Avenue, Cambridge, CB3 0FF, UK
Academic Editor:Batia Kaplan
Front. Biosci. (Landmark Ed) 2010, 15(1), 373–396;
Published: 1 January 2010
(This article belongs to the Special Issue Protein deposition diseases: pathology and micro-analytical methods)
Abstract

Protein misfolding and aggregation are implicated in a wide range of increasingly prevalent human diseases ranging from dementia to diabetes. In this review we discuss the current experimental strategies that are being employed in the investigation of the pathogenesis of three important protein misfolding disorders. The first, Alzheimer's disease (AD), is the most prevalent neurodegenerative disease and is thought to be initiated by the aggregation of a natively unstructured peptide called amyloid beta (Abeta). We discuss methods for the characterization of the aggregation properties of Abeta in vitro and how the results of such experiments can be correlated with data from animal models of disease. We then consider another form of amyloidosis, where a systemic distribution of amyloid deposit is caused by aggregation and deposition of mutational variants of lysozyme. We describe how experiments in vitro, and more recently in vivo, have provided insights into the origins of this disease. Finally we outline the varied paradigms that have been employed in the study of the serpinopathies, and in particular, a dementia caused by neuroserpin polymerization.

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