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Updated: May 2, 2026
Isolation of Soluble and Insoluble PrP Oligomers in the Normal Human Brain
Published on: October 3, 2012
Regional distribution of anchorless prion protein, PrP226*, in the human brain
Anja Lukan1, Maja Černilec1, Tanja Vranac1
1Department for the Production of Diagnostic Reagents and Research; Blood Transfusion Centre of Slovenia; Ljubljana, Slovenia.
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 PrP(Sc).
Insights
Truncated prion protein fragments (PrP226*) accumulate in specific brain regions, particularly the cerebellum, in Creutzfeldt-Jakob disease patients. This distribution mirrors that of the disease-associated prion protein (PrPSc).
Area of Science:
- Neuroscience
- Biochemistry
- Pathology
Background:
- Truncated prion protein molecules, such as PrP226*, are found in transmissible spongiform encephalopathy brains.
- PrP226* is a fragment of prion protein, truncated at Tyr226, and exists in aggregates.
Purpose of the Study:
- To investigate the distribution of the PrP226* fragment within the human brain of Creutzfeldt-Jakob disease patients.
- To determine if PrP226* distribution correlates with PrPSc distribution.
Main Methods:
- Utilized the monoclonal antibody V5B2, which specifically recognizes the PrP226* fragment.
- Analyzed the distribution of PrP226* across different human brain regions in affected individuals.
Main Results:
- PrP226* is not uniformly distributed throughout the human brain in Creutzfeldt-Jakob disease.
- The cerebellum showed the highest accumulation of the PrP226* fragment among analyzed regions.
- The distribution pattern of PrP226* closely correlated with the distribution of PrPSc.
Conclusions:
- PrP226* accumulation is region-specific within the human brain during Creutzfeldt-Jakob disease.
- The cerebellum is a key site for PrP226* aggregation.
- PrP226* distribution serves as a potential indicator for PrPSc localization in the brain.
