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

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

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

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.“

1982 Prusiner isolated a prion prions became a public interest after epidemic in U.K. 1996 - discovered a new variant of Creutzfeldt-Jacob disease

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)

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

STRUCTURE OF PRION PROTEIN AND PRION

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

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

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

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

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

Brain tissue in cattle affected by BSE

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

Thank you for your attention