(Bovine spongiform encephalopathy)

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Presentation transcript:

(Bovine spongiform encephalopathy) BSE (Bovine spongiform encephalopathy)

Structure What is BSE? Cause Infections in Humans Geographic Distribution & Prevention Diagnostic Test Summary References

1. What is BSE? BSE (Bovine spongiform encephalopathy)= mad cow disease was first reported in the United Kingdom in the 1980s symptoms: incoordination, nervous, violent, has trouble walking or getting up once the symptoms develop always progressive and fatal incubation period: 2-8 years

1. What is BSE? member of the transmissible spongiforme encephalopathies (TSEs) prion diseases progressive encephalopathies affect brain and nervous system spongy degeneration in brain and spinal cord amyloid plaques Brain from a healthy cow, as seen under a microscope using special stains. Brain from a cow sick with BSE, as seen under a microscope using special stains.

2. Cause first reported in the United Kingdom in the 1980s caused by recycling of ruminant proteins 1979 changed parameter in meat and bone meal production 20 min, 133°C, 3 bar 20 min, 80°C, 3 bar diseased sheeps were used to produce meat and bone meal (“Scrapie“) “Scrapie“ symptoms: itching, excessive lip smacking, convulsing collapse Transmissible spongiform encephalopathy (TSE)

2. Cause Prion Hypothesis responsible for the transmissible spongiforme encephalopathies (TSEs) prion in the Scrapie form (PrPSc) is an infectious agent composed of protein in a misfolded form “PrP + PrPSc  2 PrPSc“ (refolding of native proteins in diseased state) consequences: exponentially increase, tissue damage, cell death, amyloid plaques PrP PrPSc 3 x α-helix 2 x α-helix 2 x β sheet 4 x β sheet

3. Infections in humans infection: eating contaminated food variant Creutzfeld-Jakob disease (vCJD) incubation period: 11 to 12 years median age of onset: 26 years symptoms: psychiatric symptoms, incoordiantion, memory loss, persistent painful sensory symptoms, slurred speech no treatment available, only supportive care Spontaneous (genetic) Creutzfeld-Jakob Disease in humans (CJD) variant Creutzfeld-Jakob Disease (vCJD) Kuru Disease BSE

3. Infections in humans Spontaneous (genetic) Creutzfeld-Jakob Disease in humans (CJD) variant Creutzfeld-Jakob Disease (vCJD) Kuru Disease BSE Creutzfeld-Jakob Disease (CJD) Sporadic CJD: develops spontaneously accounts for 85 percent of cases first appears between ages 60 and 65 Familial CJD: heredity form caused by changes in the prion protein gene genetic changes are "dominant” accounts for about 10 to 15 percent of cases

3. Infections in Humans Spontaneous (genetic) Creutzfeld-Jakob Disease in humans (CJD) variant Creutzfeld-Jakob Disease (vCJD) Kuru Disease BSE Kuru Disease among members of the Fore tribe of Papua New Guinea caused by cannibalism Affected especially women and children of the tribe

4. Geographic distribution & Prevention first reported in the United Kingdom in the 1980s 2006: 99 cases 2008: 35 cases 2009-2011: 7 – 11 cases each year Cases of BSE: European countries, Canada, in the U.S., Israel, Japan Countries free of BSE: Iceland, Australia, New Zealand

4. Geographic Distribution & Pervention Prevention: 2001: feed ban and active surveillance for BSE in the E.U. 2001: over the age of 30 months 2008: over 48 months 2011: over 72 months 2013: no obligation anymore Germany: over 96 months 2010-2013: 3 306 579 animals tested  0 animals infected

5. Diagnostic Tests For BSE in Cattle no live animal test for BSE diagnosed by detecting prions in the CNS: polyacrylamide gel electrophoresis (proteins seperated) Proteolysis by adding protease K Western blotting (detect prion)

6. Summary BSE is a relatively new disease fatal neurodegenerative disease caused by a prion result of recycling ruminant proteins humans can be infected (vCJD) prevention: surveillance programs and not feeding meat and bone meal

7. References http://www.cfsph.iastate.edu/Factsheets/pdfs/bovine_spongiform_encephalopathy.pdf http://www.alz.org/dementia/creutzfeldt-jakob-disease-cjd-symptoms.asp http://www.bmel.de/DE/Tier/2_Tiergesundheit/Tierseuchen/_texte/BSE-FaelleDeutschland.html Dumpitak, C.; Riesner, D. Der Beweis der Prionenhypothese. Biologie in unserer Zeit. 2005, 35, 374‐383 http://www.biologie.uni-duesseldorf.de/Institute/Physikalische_Biologie/Research/Topics/addinformation2 http://www.vis.bayern.de/ernaehrung/verbraucherschutz/tiergesundheit/bseklinik.htm