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Reflections on amyloidosis in Papua New Guinea
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Genetics and Pathology.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Genetics and Pathology.
2008 (English)In: Philosophical Transactions of the Royal Society of London. Biological Sciences, ISSN 0962-8436, E-ISSN 1471-2970, Vol. 363, no 1510, 3701-3705 p.Article, review/survey (Refereed) Published
Abstract [en]

The amyloidoses comprise a heterogeneous group of diseases in which 1 out of more than 25 human proteins aggregates into characteristic beta-sheet fibrils with some unique properties. Aggregation is nucleation dependent. Among the known amyloid-forming constituents is the prion protein, well known for its ability to transmit misfolding and disease from one individual to another. There is increasing evidence that other amyloid forms also may be transmissible but only if certain prerequisites are fulfilled. One of these forms is systemic AA-amyloidosis in which an acute-phase reactant, serum AA, is over-expressed and, possibly after cleavage, aggregates into amyloid fibrils, causing disease. In a mouse model, this disorder can easily be transmitted from one animal to another both by intravenous and oral routes. Also, synthetic amyloid-like fibrils made from defined small peptides have this property, indicating a prion-like transmission mechanism. Even some fibrils occurring in the environment can transmit AA-amyloidosis in the murine model. AA-amyloidosis is particularly common in certain areas of Papua New Guinea, probably due to the endemicity of malaria and perhaps genetic predisposition. Now, when kuru is disappearing, more interest should be focused on the potentially lethal systemic AA-amyloidosis.

Place, publisher, year, edition, pages
2008. Vol. 363, no 1510, 3701-3705 p.
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-103615DOI: 10.1098/rstb.2008.0073ISI: 000260864500045PubMedID: 18849285OAI: oai:DiVA.org:uu-103615DiVA: diva2:218573
Note
Conference Information: Conference on End of Kuru - 50 Years of Research into an Extraordinary Disease Royal Soc, London, ENGLAND, OCT 11-12, 2007 Available from: 2009-05-20 Created: 2009-05-20 Last updated: 2017-12-13Bibliographically approved

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