1. Petra Jenišová Veronika Plačková Magdaléna Trojanová
PRIONS PRION DISEASES
Petra Jenišová
Veronika Plačková
Magdaléna Trojanová

2. Prions infectious proteins pathogenic form long incubation period
occur in nerve cells of mammals
cause diseases of the nervous system

3. Prion proteins created with a gene located on 20 humans chromosome
on the surface of neurons (nerve cells)
applied at rest and sleep

4. History 1939 - possibility of transmission of scrapia
1954- scrapia was marked as a slow viral infection
1976 Carlton Gajdusek-Nobel prize for contribution to the study of Curu
S. B. Prusiner: „Agent of infectious disease is a protein.“

5. 1982 Prusiner isolated a prion
prions became a public interest after epidemic in U.K.
discovered a new variant of Creutzfeldt-Jacob disease

6. Prion theory prions are defective form of normal prion protein
the primary structure of prion and prion protein are identical
the difference between them is in the secondary structure
prion is actually a prion protein with a different spatial arrangement (configuration)

7. very resistant to physical agents (temperature, low pH, UV radiation)
the ability to resist proteazam (enzymes that are capable of cleave proteins)
able to attach on the healthy form of the prion protein and converted to its defective form

8. STRUCTURE OF PRION PROTEIN AND PRION

9. Change of prion protein in prion
Mutation of a gene
change in the structure of DNA
Exchange of amino acid
Contact of prion protein and prion
prion has the ability to change prion protein in prion

10. Prion diseases Human diseases Kuru Creutzfeldt – Jacob disease
Animal diseases
Bovine spongiform encephalopathy (BSE)
Scrapie

11. Kuru discovered after World War II on New Guinea
symptoms: dizziness, weakness, headache, cramps in legs
Carleton Gajdusek examined the brains of victims and created the theory of „slow virus“ (Nobel prize)
diffused by ritual cannibalism

12. Creutzfeldt–Jacob disease
Alfons Maria Jacob, Hans Gerhard Creutzfeldt
usually affects people in the age around 55
symptoms: -personally changes
-sclerosis
-progressive dementia
-coordination dysfunction

13. Bovine spongiform encephalopathy (BSE)
„mad cow disease“
diagnosed in 1986
in the late stage of disease unusual behaviour
make pores in the brain
long incubation period

14. Brain tissue in cattle affected by BSE

15. Scrapie the longest known prion disease
the first mention from Britain (1732)
course of the disease is similar to BSE

16. Thank you for your attention