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Atypical Prion Protein Conformation in Familial Prion Disease with PRNP P105T Mutation

Polymenidou, Magdalini and Prokop, Stefan and Jung, Hans H. and Hewer, Ekkehard and Peretz, David and Moos, Rita and Tolnay, Markus and Aguzzi, Adriano. (2011) Atypical Prion Protein Conformation in Familial Prion Disease with PRNP P105T Mutation. Brain pathology, Vol. 21, no. 2. pp. 209-214.

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Official URL: http://edoc.unibas.ch/dok/A6005164

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Abstract

Protease-resistant prion protein (PrP(Sc) ) is diagnostic of prion disease, yet its detection is frequently difficult. Here, we describe a patient with a PRNP P105T mutation and typical familial prion disease. Brain PrP(Sc) was undetectable by conventional Western blotting and barely detectable after phosphotungstate precipitation, where it displayed an atypical pattern suggestive of noncanonical conformation. Therefore, we used a novel misfolded protein assay (MPA) that detects PrP aggregates independently of their protease resistance. The MPA revealed the presence of aggregated PrP in similar amounts as in typical sporadic Creutzfeldt-Jakob disease. These findings suggest that measurements of PrP aggregation with the MPA may be potentially more sensitive than protease-based methodologies.
Faculties and Departments:03 Faculty of Medicine > Bereich Querschnittsfächer (Klinik) > Pathologie USB > Allgemeine Pathologie (Tolnay)
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Querschnittsfächer (Klinik) > Pathologie USB > Allgemeine Pathologie (Tolnay)
UniBasel Contributors:Tolnay, Markus
Item Type:Article, refereed
Article Subtype:Research Article
Bibsysno:Link to catalogue
ISSN:1015-6305
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:27 Mar 2014 13:13
Deposited On:27 Mar 2014 13:13

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