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UNCONVENTIONAL VIRUSES/AGENTS/PRIONS V.E. Gabriel, MD
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES (TSEs, TRANSMISSIBLE CEREBRAL AMYLOIDOSES, PRION DISEASES)
 
 
 
 
 
 
 
 
 
 
<ul><ul><ul><li>Stanley B. Prusiner  coined the term  proin from  Pro teinaceous  in fective particle </li></ul></ul></ul>...
<ul><li>Kuru </li></ul><ul><li>Fatal Familial Insomnia (FFI) </li></ul><ul><li>Creutzfeldt-Jakob disease (CJD) </li></ul><...
Classification of prion diseases <ul><li>Infectious/Exogenous </li></ul><ul><ul><li>e.g., Kuru, BSE (mad cow disease), Scr...
Differences between cellular and scrapie proteins <ul><ul><ul><li>PrP C PrP SC </li></ul></ul></ul><ul><ul><ul><li>Solubil...
Gross and Microscopic Changes  <ul><ul><ul><li>Grossly there is Cortical atrophy  and  ventricular  dilatation may also be...
<ul><li>Microscopic changes </li></ul>Scrapie BSE CJD Kuru
<ul><ul><ul><li>Gliosis within plaques. </li></ul></ul></ul><ul><ul><ul><li>Loss of oligodendrocytes within plaques. </li>...
<ul><ul><ul><li>Diagnosis can be made by: </li></ul></ul></ul><ul><ul><ul><li>1. Clinical signs and Symptoms.  </li></ul><...
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39. unconventional viruses

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39. unconventional viruses

  1. 1. UNCONVENTIONAL VIRUSES/AGENTS/PRIONS V.E. Gabriel, MD
  2. 2. TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES (TSEs, TRANSMISSIBLE CEREBRAL AMYLOIDOSES, PRION DISEASES)
  3. 13. <ul><ul><ul><li>Stanley B. Prusiner coined the term proin from Pro teinaceous in fective particle </li></ul></ul></ul><ul><ul><ul><li>and changed to prion to sound it rhythmic. </li></ul></ul></ul><ul><ul><ul><li>Prion diseases were caused by misfolded proteins. </li></ul></ul></ul><ul><ul><ul><li>Elucidated the gene and mechanism by which wild type protein bring about the </li></ul></ul></ul><ul><ul><ul><li>clinical disease. </li></ul></ul></ul>INTRODUCTION
  4. 14. <ul><li>Kuru </li></ul><ul><li>Fatal Familial Insomnia (FFI) </li></ul><ul><li>Creutzfeldt-Jakob disease (CJD) </li></ul><ul><li>Scrapie </li></ul><ul><li>Bovine Spongiform Encephalopathy (BSE) </li></ul><ul><li>Chronic Wasting Disease (CWD) </li></ul>Prion Diseases Human Animal
  5. 15. Classification of prion diseases <ul><li>Infectious/Exogenous </li></ul><ul><ul><li>e.g., Kuru, BSE (mad cow disease), Scrapie </li></ul></ul><ul><ul><li>Spread by </li></ul></ul><ul><ul><ul><li>Consumption of infected material. </li></ul></ul></ul><ul><ul><ul><li>Transfusion. </li></ul></ul></ul><ul><li>Sporadic </li></ul><ul><li>Familial/Hereditary </li></ul><ul><ul><li>Due to autosomal dominant mutation of PrP. </li></ul></ul>
  6. 16. Differences between cellular and scrapie proteins <ul><ul><ul><li>PrP C PrP SC </li></ul></ul></ul><ul><ul><ul><li>Solubility </li></ul></ul></ul><ul><ul><ul><li>Soluble Non soluble </li></ul></ul></ul><ul><ul><ul><li>Structure </li></ul></ul></ul><ul><ul><ul><li>Alpha-helical Beta-sheeted </li></ul></ul></ul><ul><ul><ul><li>Multimerisation state </li></ul></ul></ul><ul><ul><ul><li>Monomeric Multimeric </li></ul></ul></ul><ul><ul><ul><li>Infectivity </li></ul></ul></ul><ul><ul><ul><li>Non infectious Infectious </li></ul></ul></ul><ul><ul><ul><li>Susceptibility to Proteinase K </li></ul></ul></ul><ul><ul><ul><li>Susceptible Resistant </li></ul></ul></ul>
  7. 17. Gross and Microscopic Changes <ul><ul><ul><li>Grossly there is Cortical atrophy and ventricular dilatation may also be present. </li></ul></ul></ul><ul><li>Gross changes </li></ul>
  8. 18. <ul><li>Microscopic changes </li></ul>Scrapie BSE CJD Kuru
  9. 19. <ul><ul><ul><li>Gliosis within plaques. </li></ul></ul></ul><ul><ul><ul><li>Loss of oligodendrocytes within plaques. </li></ul></ul></ul><ul><ul><ul><li>Axons usually remain intact in plaques. </li></ul></ul></ul><ul><ul><ul><li>Both CD4+ and CD8+ lymphocytes are present in active </li></ul></ul></ul><ul><ul><ul><li>lesions. </li></ul></ul></ul>Other microscopic changes
  10. 20. <ul><ul><ul><li>Diagnosis can be made by: </li></ul></ul></ul><ul><ul><ul><li>1. Clinical signs and Symptoms. </li></ul></ul></ul><ul><ul><ul><li>2. Detection of Scrapie </li></ul></ul></ul><ul><ul><ul><li>Associated fibrils. </li></ul></ul></ul><ul><li>3. Detection of Abnormal Prion protein (PrP sc ) by Western blotting. </li></ul><ul><li>4. Two dimensional Gel Electrophoresis. 5. Imunodiagnosis of Prion disease. </li></ul><ul><li>6. Bioassay in Mice. </li></ul>Diagnosis <ul><ul><ul><li>Scrapie Associated fibrils. </li></ul></ul></ul>
  11. 21. Thank You

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