Projects / Programmes source: ARIS

Prions in human medicine: from structural studies to applications

Research activity

Code Science Field Subfield
3.01.00  Medical sciences  Microbiology and immunology   

Code Science Field
B007  Biomedical sciences  Medicine (human and vertebrates) 
prion protein, prions, monoclonal antibodies, diagnostics, therapy, decontamination
Evaluation (rules)
source: COBISS
Researchers (12)
no. Code Name and surname Research area Role Period No. of publicationsNo. of publications
1.  25730  PhD Anja Colja Venturini  Pharmacy  Researcher  2008 - 2011  17 
2.  14136  MSc Maja Černilec  Microbiology and immunology  Researcher  2008 - 2011  51 
3.  05236  PhD Vladka Čurin Šerbec  Microbiology and immunology  Head  2008 - 2011  261 
4.  06416  PhD Marko Dolinar  Biochemistry and molecular biology  Researcher  2008 - 2011  340 
5.  29079  PhD Andrej Hanzlowsky  Biochemistry and molecular biology  Researcher  2008 
6.  15978  PhD Katrina Hartman  Microbiology and immunology  Researcher  2008 - 2011  43 
7.  25973  PhD Simon Koren  Biochemistry and molecular biology  Researcher  2008 - 2011  88 
8.  28420  PhD Miha Kosmač  Microbiology and immunology  Junior researcher  2008 - 2011  13 
9.  10873  PhD Nataša Poklar Ulrih  Chemistry  Researcher  2008 - 2011  820 
10.  28444  Nives Škrlj  Biotechnology  Junior researcher  2008 - 2011  14 
11.  30763  PhD Marko Šnajder  Biotechnology  Junior researcher  2009 - 2011  39 
12.  21335  PhD Tanja Vranac  Biochemistry and molecular biology  Researcher  2008 - 2011  40 
Organisations (3)
no. Code Research organisation City Registration number No. of publicationsNo. of publications
1.  0103  University of Ljubljana, Faculty of Chemistry and Chemical Technology  Ljubljana  1626990  23,093 
2.  0311  Blood Transfusion Centre of Slovenia  Ljubljana  5053960  1,747 
3.  0481  University of Ljubljana, Biotechnical Faculty  Ljubljana  1626914  65,921 
Prion diseases and prions, which are supposed to be unusual infectious agents, are under scientific and public attention since the eightees, when the epidemics of bovine spongiform encephalopathy (BSE) arose in the United Kingdom (UK). The first case of variant Creutzfeldt-Jakob Disease (vCJD) was reported ten years later in the same country. Transmission from cattle to humans probably occured due to the consumption of BSE infected meat and meat products, possibly also with vaccines or drugs, which had bovine proteins in their formulations. Transmission of the disease by blood transfusion from infected asymptomatic blood donors has been reported in the last years and emphasized the importance of basic and applicative research in the field of prion science.Research projects thus focus on the development of sensitive methods for premortem diagnostics with new technologies, on the development of therapy and prevention and on the development of succesful procedures of decontamination and desinfection. It is also very important to know more about the structure of prions to be successful in all above mentioned activities. With the project "Prions in human medicine: from structural studies to applications" we'll continue the research started in the scope of previous projects. We intend to study the presence of the two isoforms of prion protein, cellular (PrPC) and pathological (PrPSc), on/in different subpopulations of human blood cells and in body fluids. Our studies will include healthy population as well as patients with suspected CJD and confirmed CJD. A panel of monoclonal antibodies (mAbs) will be used, specially previously described PrPSc-specific mAb V5B2. Anti-idiotypic monoclonal antibodies against mAb V5B2 will also be prepared and V5B2 will be humanised. With both approaches we’ll enroll in the field of active and passive protection against prion diseases. Amino acids, crucial for the binding of peptide P1 to mAb V5B2, will be determined by different approaches: by using synthetic peptides, by phage peptide display and by crystallographic studies. A new generation of mAbs, similar to V5B2 will be prepared in order to react selectively with mouse and hamster PrPSc. They will be very useful tool for different studies using animal models. Structural studies of extracellular proteins from A.pernix will also be performed, since these proteins totally digest prions. Recombinant proteins will be prepared, as well, in order to install biotechnological process in bioreactor for the protein aggregates degradation and cosequent production of energy.
Significance for science
The project team has shown with the results in the past years to be capable to carry out demanding research and applicative projects. In the scope of this project, some still unsolved problems in the field of prion biology are studied with new tools, which we have developed. Some of them are implemented in diagnostic and/or therapeutic purposes not only in the field of prions, but also in other medical fields. We would also like to explore the possibility for decontamination of waste , containing infective protein aggregates and consequent use of energy, released in this process. The number of publications and their citations, articles in press or sent for publication, the invitation to write a review article and a book chapter, granted and filled patents as well as co-financing of from the foreigh industrial partner confirm the quality of our work in the field of neurodegenerative disorders, which is supposed to be one of the most difficult in biomedicine.
Significance for the country
On its progress among the most developed European countries, Slovenia needs groups, which are able to carry out applicative projects and implement the results into routine use besides publishing research articles in renowned journals. The field of biomedicine, which brings together life sciences (biochemistry and molecular biology), medical sciences (immunology, transfusion medicine) and biotechnology, is a good example. Development of new tools and new technologies is the basis for a modern and successful European country, which in the long term assures well-being of its citizens. It also enables equal involvement in global issues and representation of the country in the world's most important professional and scientific organisations. Last, but not least, highly educated professionals for industry and other public institutions are raised during our research processes. The gained knowledge also represents the basis for new employments and start up of new companies.
Most important scientific results Annual report 2008, 2009, final report, complete report on dLib.si
Most important socioeconomically and culturally relevant results Annual report 2008, 2009, final report, complete report on dLib.si
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