2. What are Prions?
They are abnormal proteins
called prion proteins
pathogenic agents
Normal prion proteins
Abundantly in the brain.
Functions are not completely understood.
3. .
In the1950s
New Guinea
Fatal disease on people of the Fore tribe.
“Kuru” "trembling in fear."
Becoming unable to walk,
Lost the ability to swallow or chew.
Drastic weight loss
Inevitably lead to death.
Prions are the responsible.
4. Prion diseases
A family of rare progressive neurodegenerative
disorders
Affecting humans and animals.
They are distinguished by:
long incubation periods,
characteristic spongiform changes associated
with neuronal loss,
Failure to induce inflammatory response.
Normal brain tissue Spongiform encephalitis
5. The abnormal folding
of the prion proteins
leads to brain
damage and the
characteristic signs
and symptoms of the
disease.
9. Prion disease
Affect the nervous system.
In people:
Impair brain function,
Changes in memory, personality, and behavior;
Decline in intellectual function (dementia);
Abnormal movements, difficulty with coordinating
movements (ataxia).
Typically starts in adulthood and worsen with time,
Leading to death within a few months to several
years.
Approximately 350 new cases are reported
annually in the United States.
10. Causes of prion diseases
10 and 15% by mutations in the
PRNP gene.
85 to 90 % are sporadic or
acquired.
Acquired results from exposure to
PrPSc from an outside source.
from eating beef products containing PrPSc
Individuals ate affected human tissue during
cannibalistic funeral rituals.
Iatrogenic. 1 to2% can be
transmitted by accidental
exposure to PrPSc-contaminated
tissues during a medical
procedure.
12. Bibliography
The Johns Hopkins University. (2019) Prion Diseases.
Retrieved from https://www.hopkinsmedicine.org/health/conditio
ns-and-diseases/prion-diseases
Centers for Disease Control and Prevention. (2019) Prion Diseases.
Retrieved from https://www.cdc.gov/prions/index.html
