Regional distribution of anchorless prion protein, PrP226, in the human brain

It was shown previously that truncated molecules of prion protein can be found in brains of patients with some types of transmissible spongiform encephalopathy. One such molecule, PrP226*, is a fragment of prion protein, truncated at Tyr226. It was found to be present in aggregates, from which it ca...

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Bibliographic Details
Published in:Prion 2014, Vol.8 (2), p.203-209
Main Authors: Lukan, Anja, Černilec, Maja, Vranac, Tanja, Popović, Mara, Čurin Šerbec, Vladka
Format: Report
Language:English
Subjects:
anchorless PrP
denaturation
immunoassay
mAb V5B2
PrP fragment
PrP226
regional distribution
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Summary:It was shown previously that truncated molecules of prion protein can be found in brains of patients with some types of transmissible spongiform encephalopathy. One such molecule, PrP226*, is a fragment of prion protein, truncated at Tyr226. It was found to be present in aggregates, from which it can be released using chaotropic salts. In this study we investigated the distribution of PrP226* in Creutzfeldt-Jakob disease affected human brain, employing the mAb V5B2, specifically recognizing this fragment. The results show that PrP226* is not evenly distributed among different regions of human brain. Among brain regions analyzed, the fragment was found most likely to be accumulated in the cerebellum. Its distribution correlates with the distribution of PrP Sc .
ISSN:1933-6896
1933-690X
DOI:10.4161/pri.28388