The Connection

1
The Connection

• One Thing Leads to Another
• Keary Raines

2
Genesis ( the beginning)

• Native Kuru diagnosis (1956)
• Gajdusek discovers Fore people along with Kuru
• Brain examinations
• Results in the suspicion of cannibalism

3
Scrapie

• Scrapie (1930s)
• French vets identify scrapie as infectious
• Injected homogenized scrapie into healthy animals
• Detailed Scrapie examination (1950s)
• Bill Hadlow finds glial cells (astorgliosis) in
  sheep brains
• Careful examination of brain slices under
  microscope

4

• Georgette (1965)
• Joe Gibbs (and scientists) show transmissible
  agent underlies Kuru
• Transferred Kuru to Georgette
• Scrapie resistance
• D.R. Wilson shows that scrapie survives nearly
  anything
• Numerous attempts stop transmissibility

5
DNA?

• Agent size (1960s)
• Alpor, Haig, Clark say that scrapie has no DNA
• Electron bombardment, UV light
• Strains (1960s)
• Alan Dickenson says Scrapie has DNA in protein
  shell
• 22C/22A observation

6
Connection to Alzheimers

• Sticks
• Patricia Merz discovers scrapie-associated
  fibrils (SAF)
• Electron microscope
• PrP (1984)
• Stanley Prusiner found PrP in healthy and
  infected sheep tissues

7
More Protein Evidence

• Conversion (1995)
• Richard Bessen change good protein into abnormal
  forms
• Mixed normal protein with two different strains
  of abnormal PrP

8
vCJD and BSE

• Confirmed (1997)
• Dr. Moira and scientist confirm that vCJD is
  human form of BSE
• Inoculation of mice with BSE, TSE, and vCJD

9
Scientific Patterns

• Susan Wright

10
Patterns

• Represent similarities between two unknowns
• Patterns provide a logical example
• Link the work of different scientists

11
Kochs Postulates

• Agent must be present in every case
• Inoculations of pure cultures must produce
  disease in animals
• Cultures purified from diseased animals must
  reproduce the disease

12
Inoculation Patterns

• Gajdusek inoculated mice with scrapie
• Gibbs inoculated chimps with kuru
• French infected sheep with scrapie

13
Brain Examinations

• Amyloid plaques in kuru brain
• Astrgliosis of CJD infected brain
• Spongiform change in mink brain
• Severe status spongiosus in Georgettes brain

14
Symptom Stage Patterns

• Prion diseases share common stages of
  degeneration
• Ambulant unsteadiness, speech deterioration,
  tremors
• Sedentary ataxia, depression, cannot walk
  without support
• Terminal urinary incontinence, dysphagia,
  cerebellar dysfuntion

15
The Scientist of Slow Growing Diseases

• Courtney Hahn

16
D. Carleton Gajdusek
17
Younger Years

• Was born on September 9, 1923
• Worked in Boyce Thompson Laboratories during
  summers thirteen to sixteen
• Synthesized acids becoming weed killers with a
  patent

18
Later School Years

• Studied at University of Rochester from 1940 to
  1943
• Spent four years at Harvard Medical School
• Spent one year at Caltech
• Studied at the Institute Pasteur of Teheran

19
Australia

• Went to Australia in 1954 for the Institute of
  Medical Research in Melbourne
• Studied immunology and virology
• Started child development and disease patterns
  studies

20
Kuru

• Saw kuru for first time in 1957
• Joined NIH in 1958
• Worked in Iran, Australia and U.S studying
  infectious diseases
• Won Nobel Prize for work on kuru in 1976

21
Stanley B. Prusiner
22
Early School

• Born in Des Moines, May 28, 1924
• Studied Latin five years at Walnut Hills High
  School
• Majored in Chemistry at University of
  Pennsylvania
• Studied hypothermia in Department of Surgery
  summer of 1963

23
College

• Attended Penn for Medical School
• Studied brown adipose tissue at Wenner-Gren
  Institute in Stockholm
• Intern at University of San Francisco
• Worked at the NIH for three years
• Residency at University of California San
  Francisco, 1972

24
Claim to Fame

• Female patient diagnosed with CJD
• Began studying scrapie in 1974
• Introduced prion in 1982
• Prion was found in laboratory in 1983
• Won Nobel Prize 1996

25
Personalities

• Show hard work and perseverance through years of
  school
• Also show patience when working with slow growing
  diseases
• Both show compassion to humans with brain
  diseases

26
Social Interaction

• Galo Bueno

27
Primary Scientists

• Dr. Gajdusek
• Dr. Hadlow
• Dr. Creutzfeldt
• Dr. Jakob

• Dr. Brown
• Dr. Smadel
• Dr. Gibbs
• Dr. Pursiner

28
Communication

• Scientists went to find help and information from
  other scientists any time they need results.
• Jakob and Creutzfeldt teamed up and worked on
  curing Creutzfeldt-Jakob disease.
• Gadjdusek and Hadlow worked on scrapie.
• In different cases scientists helped each other
  gather information.

29
Positive Communication

• Creutzfeldt and Jakob discovered the disease
• Gajdusek recruited Brown from New Guinea to help
  him in his research
• Hadlow and Gajdusek worked on the scrapie disease

30
Negative Communication

• Merzs and Prusiner did not agree on Prions
• With no communication between scientists there
  cannot be developments in new cures.

31
Vivisection

• Kyle Kocourek

32
Vivisection What is it?

• The act of operating on living animals.
• Especially in scientific experiments.
• Literal meaning cutting while still alive

33
Pros and Cons

• Pros
• Does not hurt humans
• Test theories on animals rather than people

• Cons
• Animals not strongly related to human qualities
• Ethics

34
Experiments

• From text
• Primate inoculation of scrapie
• Georgette
• Mink
• Mice
• Goats
• Sheep
• Chimps with kuru and CJD

35
Experiments

• Outside of Text
• Pig-to-primate organ transplant experiments
  conducted by Imutran at Huntingdon Life Sciences
• Pig hearts and kidneys transplanted in necks and
  abdomens of hundreds of monkeys
• Then administered lethal doses of
  immunosuppressants in a futile attempt to prevent
  rejection.

36
Sources

• http//www.uncaged.co.uk/vivisect.htm
• www.wikipedia.org
• www.nobelprize.org
• Deadly Feast by Richard Rhodes