Author/Editor     Lukan, Anja; Černilec, Maja; Vranac, Tanja; Popović, Mara; Čurin-Šerbec, Vladka
Title     Regional distribution of anchorless prion protein, PrP226*, in the human brain
Type     članek
Publication year     2014
ISSN     1933-6896 - Prion
Language     eng
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.
Keywords     prion protein
human brain
Creutzfeldt-Jakob disease
prionski protein
človeški možgani
Creutzfeldt-Jakobova bolezen