Structure of

Prion
The normal prion structure is
characterized by four helices, denoted as
PrPc
The abnormal one consist of 2 helices
and 4 beta-sheets, which derived from the
two helices of PrPc, is denoted as PrPse
Figure 1: Proposed three-dimensional structure (a) PrPC and (b)
PrPSc

The Conversion of PrP
c
to PrP
se
(a)
(b)
Chain-reaction Conversion of PrPc to
PrPsc
PrPc PrPsc Chain-reaction Conversion
The harmful PrPSc form is very resistant to high temperatures,
UV-irradiation and strong degradative enzymes.
How PrPsc attack the Brain?
Brain consists of a mass of nerve tissue (Hundred billions of
neuron) and neuroglia, supporting neural tissue.






When enough PrPSc proteins have been made they form long
filamentous aggregates that gradually damage neuronal tissue.

When neuron in the brain are all dead, the appearance of the
brain will become sponge-like appearance. And this eventually
lead to death.

Different prions affect different regions of the
brain

Cerebral cortex: the symptoms include
loss of memory and mental acuity, also visual
imparement (CJD).









Thalamus: Fatal Familial Insomnia (FFI).
Cerebellum: lose the control of body
movements and difficulties to walk (kuru,
GSS).

Brain stem: In the mad cow disease (BSE),
the brain stem is affected.

Prions infect animals &
Humans
What are the Prion-Related Diseases?





Are all known prion diseases
fatal ?
YES, they are.
& where Prion-Related Diseases Occur?

TSEs
T: Transmissible (Communicable)
S: Spongiform (Look like sponge)
E: Encephalopathy (Brain diseases)

Prion diseases are often called transmissible
spongiform encephalopathies (TSE).
Because after the infection, the brain
appearance become a sponge-like apearance.
Six forms of TSEs:











1) CJD - Creutzfeld-Jacob Disease
2) BSEs -Bovine-Spongiform-
Encephalopathy
3) Alper's Syndrome

4) GSS - Gerstmann-Straussler-
Scheinkner syndrome
5) FFI - Fatal Familial Insomnia

6) Kuru


CJD - Creutzfeld-Jakob Disease
The disease causes fatal degradation of brain tissue and
produces a dementia that affects men and women, often
between the ages of 50 and 65.






90% cases progress to death within a year

There is no record of anyone recovering from the
disease and there is no known treatment.

Causes
CJD is associated with prions, mutated forms of a
normal protein produced in nerve cells, white
blood cells, muscle cells, and the cells of many
other tissues.

Just how prions cause the disease symptoms
remains unclear.

There are three types of the disease: sporadic,
genetic, and iatrogenic.

The 3 Type of CJD
Sporatic CJD: Spontaneous mutation. 85% of the cases.
genetic CJD: a mutant prion gene is passed genetically from one
generation to another. 10-15% of the cases.
Iatrogenic CJD: Contaminated directly to the brain (eg: Surgery)


New variant CJD: CJD with shorter incubation: a form of
Creutzfeldt-Jakob disease that has a much shorter incubation period
than previously recognized types but is clinically identical. It first
appeared in the late 1980s.
BSEs: Bovine Spongiform
encephalopathy
BSE
Way of infection












The cow eat offal of the infected sheep
Prions are taken up from the gut and transported
along nerve fibers to the brain stem.
Prions accumulate and convert normal prion
proteins to the disease-causing form, PrPSc.
Years later, BSE results when a sufficient number of
nerve cells have become damaged, affecting the
behaviour of the cows. And eventually the cow is dead.
Symptom
Bored with food, unsociable .
No capability in memorizing anything..
Loss of control of the muscle movement.
Can not speak and swallow the food.