Prions are abnormal protein particles that can cause neurodegenerative diseases. They have a normal function in the body but can transform into an infectious form. When this happens, they induce other normal prion proteins to adopt the abnormal shape. Common prion diseases include scrapie in sheep, mad cow disease, and Creutzfeldt-Jakob disease in humans. There are currently no effective treatments for prion diseases, so prevention and diagnosis are important.

2. GUIDED BY
WINSON SAM
PROJECT BY
RAHULD

3. WHAT IS A
PRION
NORMAL
FUNCTION
OF PRION
HOW PRION
BECOME
INFECTIOUS
AGENT
DIAGNOSIS
AND
TREATMENT
PRION
DISEASE
Preventionconclusion

4. Prions are protiens that are found mostly abundant in the
brain.
They doesn’t use energy, they don’t grow, they don’t die.
They reproduce via pre-existing prions.
All the mammals have prion protein gene.
Prions have some normal fuctions in our body.
 They help in the long term memory.
 also causes the activation of mylien
repair in schwann cell.
 Prion expression in the stem
cell neccesary for self
renewal of the bone marrow.
 Antioxidant activity
 Prevent neuronal dysfunction

5. PRIONS THE infective agent
Normally prions have the α-helical structure when their
conformation changes it form β-sheets. This occurs due to
mutation in the prion gene or by infection of infective prion
from other source.
Then its changes it nature , now it is an infective protien
particle that are capable in producing diseases called prion
diseases.

6. Stanley B Prusiner in 1982
coined the word Prion which
is derived from the word
protien and infection.
Prusiner got the Nobel prize
in 1997 for his research into
prions.
Normal prions are denoted
as PrP and infective as PrPSc.
This PrPSc can change
normal prions to infective
ones.

8. Prion diseases
IN ANIMALS
Scrapie
Mad cow disease
IN HUMANS
Kuru
Creutzfeldt jacob disease
Familial insomnia
Gerstmann–Sträussler–Scheinker syndrome

9. Scrapie
First diagnosed prion disease. Found in sheep
which affect the nervous system of sheep and its
is not transmissible to humans. Clinical signs are
behavioral changes increased chewing movement
ataxia and intense itching.

10. MAD COW DISEASE
Neuro degenerative disease which caused by prions
in cows and is also known as Bovine spongiform
Encephalopathy and it infects the human beings by
the consumption of the contaminated meat and
also through its body fluids.

11. Prions disease occur in cattle
are mainly due to
feeding cattle with the meat
and bone meal of infected
sheeps.
Infected cows will be dead
within 6 months.
Clinical signs
 Incoordinated body
movement Aggression
 decreased milk production
 loss of body weight.

12. KURU
kuru is an incurable degenerative disorder
which found in the tribal region of Papua
New Guinea is a type of transmissible
spongiform encephalopathy caused by the
prions. Word kuru derives from the word
kuria means to shake and also it is known
as laughing sickness.
It orginated around 1900, and main reason
for its spread is the practice of cannibalism

13. Symptoms
 Headache, joint pain(first days)
 After 6-12 weeks Shaking of
body unsttopable laughing
 After 12 months Difficulty in
walking and speaking
 Death within the 2 years.
Nobel prize 1976 to carlton
Gadjesek for research in kuru and
reason for its transmission.

14. CRUETZFELDT JACOB DISEASE
CJD is the most dangerous Prion
disease with more death rates
than the others. CJD is also an
incurable neuro degenerative
disorder. CJD mainly affect the
cerebral cortex. There are four
different types of CJD.
 SPORADIC CJD
 IATROGENIC CJD
 GENETIC CJD
 VARIENT CJD

15. Sporadic CJD

16. Familial or genetic CJD

17. It is a very rare, usually familial, fatal neurodegenerative
disease that affects patients from 20 to 60 years in age.
A change incodon 102 from proline to leucine on
chromosome 20,
Therefore, it appears this genetic change is usually required
for the development of the disease.
Symptoms
progressive ataxia
dementia.
Gerstmann Sträussler Scheinker
syndrome

18. FATAL FAMILIAL INSOMNIA

19. DIAGNOSIS
Magnetic Resonance Imaging
(MRI)reveals the characteristics
patterns of brain degeneration
that help diagnose PrPSc
Electroencephalogram (EEG)
record the brain’s electrical
pattern which can be
particularly valuable because
it shows specific type of
abnormality in prion proteins

20. A brain biopsy done to look for spongiform change, which
would be diagnostic of sporadic CJD and A brain biopsy is a
neurosurgical procedure in which a brain surgeon removes a
small amount of brain tissue to be examined for presence of
prions.
Cerebro spinal fluid exam
In all cases of CJD , an examination of cerebrospinal fluid
should be performed. Lumbar puncture, the presence of a
particular protein called 14-3-3 in the cerebrospinal fluid is
very helpful in the diagnosis of sCJD. The increased level of
protein is caused by the rapid loss of brain cells.

21. BLOOD TEST

22. TREATMENT
Currently no drug is available for the
curing of the prion disease. And
some experimental drugs used were
Tetracycline compounds,
Quinacrine, Pentosan polysulphate,
Flupritine.
Some future drugs and
methods still under studying
are PRN-100 an antibody
And treatment with RNA
interference.

23. PREVENTION
Since the link between mad cow disease and CJD of
human was confirmed so
° A ban on feeding farm animals on meat and bone meal.
° The removal and destruction of all part of an animal
carcass infected with BSE.
Prevention of transmission of prion
disease through blood transfusion because
blood is a good carrier for the prions.
So person infectedwith prions should not
let to give blood.

24. Prion disease can also affect by organ transplant so prevent
the transplantation of the infected person.
It has proven that infected prion is not affected by normal
sterilizing procedure so instruments used for treatment of
infected person should not be used again.
There is a chance of transmission
with human growth hormone so
synthetically produced growth
hormones should be used.

25. CONCLUSION
Prion protien is an amazing
protien aswell as a devastating
disease causing agent. Prion
diseases have no treatment yet.
Prevention is the only way to
avoid prion disease. CJD is the
main disease caused from prions
and future studies for the
treatment of these disease will
lead to curing of several other
neuro degenerative disorders.

26. REFERENCE

27. THANK YOU