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Abstract
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 PrPSc.
Disclosure of Potential Conflicts of Interest
The authors declare that there are no conflicts of interest or competing financial interests.
Acknowledgments
The study was financially supported by The Slovenian Research Agency with research program P4–0176 and Ph.D. grant to A.L.
