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Acta Neuropathologica
Published in: Acta Neuropathologica 1/2011

Open Access 01-01-2011 | Review

Molecular pathology of human prion disease

Authors: Jonathan D. F. Wadsworth, John Collinge

Published in: Acta Neuropathologica | Issue 1/2011

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Abstract

Human prion diseases are associated with a range of clinical presentations and are classified by both clinicopathological syndrome and aetiology with sub-classification according to molecular criteria. Considerable experimental evidence suggests that phenotypic diversity in human prion disease relates in significant part to the existence of distinct human prion strains encoded by abnormal PrP isoforms with differing physicochemical properties. To date, however, the conformational repertoire of pathological isoforms of wild-type human PrP and the various forms of mutant human PrP has not been fully defined. Efforts to produce a unified international classification of human prion disease are still ongoing. The ability of genetic background to influence prion strain selection together with knowledge of numerous other factors that may influence clinical and neuropathological presentation strongly emphasises the requirement to identify distinct human prion strains in appropriate transgenic models, where host genetic variability and other modifiers of phenotype are removed. Defining how many human prion strains exist allied with transgenic modelling of potentially zoonotic prion strains will inform on how many human infections may have an animal origin. Understanding these relationships will have direct translation to protecting public health.
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Metadata
Title
Molecular pathology of human prion disease
Authors
Jonathan D. F. Wadsworth
John Collinge
Publication date
01-01-2011
Publisher
Springer-Verlag
Published in
Acta Neuropathologica / Issue 1/2011
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI
https://doi.org/10.1007/s00401-010-0735-5

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