Prions

Shanley
Scrapie
Prions originate from an Ancient disease called
scrapie
Seen in Sheep, goats
As their fur grow they scrap against rocks and tree but
with this disorder they scrape themselves to death, it
make it worse. When the farmer notices this he must
remove from flock because it can be passed on. They can
also notice lip smacking in goats. The is a neurological
degenerative disease.
Based on the secondary, tertiary or quaternary structure,
the protein in prion is altered and changes so you get an
abnormal structure. The folding in the protein will
change. This change will lead to prion protein. This prion
protein is self replicating, and we are not used to this.
This is usually limited to nucleic acids. The organism is
trying to produce normal protein but the prooetin is
making altered production of prion protein.
Scrape to the point of infection or death
Transmitted from infected sheep through milk
Protein folding
Protein Structure
Primary
Secondary
Tertiary
Quartinary
Altered folding
Self replicating
Protein accumulation these proteins are useless
so they will accumulate. We will see them in:
Lymph nodes
Peyers patches: small intestine
Small intestine
Nerve cells: trhoughout the entire nervous system.
The you will see large degradation because of their
presence.
Creutzfeldt-Jakob Disease (CJD)
This is a Human prion disease
The actual biologic name is Spongiform encephalopathy. This is an accumulation of prions in
the brain which will take a long time and we will see degradation of the nervous tissue. You
will see holes in the brain.
300 cases/year in US
Symptoms is late Onset later in life. These symptoms include:
Failing memory
Behavior changes
Coordination and vision changes
Mental deterioration
Involuntary ticks
Blindness
Weakness
Coma
death
Once you see the symptoms they have Death w/in 1 year
There are 3 major categories
Sporadic CJD 85% - wont see any risk factors, it is developed spontaneous. No way to predict
this
Hereditary CJD 5-10% - family related.
Acquired CJD patient is exposed to brain or nervous tissue from a person or organism with
CJD. Most cases it is through medical procedures.
This is not transmitted through casual contact, only mostly in surgery on patient that
had CJD. The medical equipment is sterilized and used again but the prions are not
destroyed. When someone is supsected of CJD we toss the equipment because the
risk is too significant.




Farming Practices and Risk
Mostly originated in UK, and Ireland
Beef farmers wanted to increase protein cattle had in feed. So they include
animal bi-products in the feed making process to increase protein. Some of
those animals were sheep. For many years the process included a
Lyophilization step where they freeze dry something. This can destroy prions.
They didnt include this step anymore and was removed from cattle feed
production, which means untreated offal (any part in the cranium and the
spine) was going to infect the cattle. As soon as the out break was discovered,
the government took the entire herd and they were left with nothing. The
farming community didnt report the cases.
The first case was Bovine spongiform encephalitis in 1986. in 1987 the
meat was already in the cattle and people are eating it. 165 people
contracted the varying CJD because it was not acquired through the
human. The protein is not a human protein and is different. the EU
band cattle from 1996 for 10 years. They acquired the disease, not
through eating a steak (even though there is a risk), but was in the
ground meat that holds the left over parts that can contain some
offal.
Transmitted to persons through ingestion of contaminated meat,
working with the cattle feed, or those who use it as a fertilizer. They
have band animal bi products and now must use soy or other things
to boost the protein in the cattle.

Iatrogenic Transmission
Kuru in the 1950 the FORE people, had kuru. Mostly affected women
and children with incoordination and balance would die within a year.
When the exam the patients at autopsy they saw that the had CJD, but
was called kuru. It was linked to funeral practices in how they
prepared the dead for burial. When the person died they were
prepared by women and children. It was common in their practice to
handle the body, including the brain. When they told the fore people
not to do it, now it is no longer transmitted and is extinct.
Acquired CJD is Transmission of infected tissue via instrumentation.
Intracerebrally of most concern including: Brain surgery, Eye surgery
and theoretically tonsil surgery (lymphs that can be affected).
Instruments should be destroyed if patient is CJD known
Autoclave procedures
First option: Immerse in pan of 1 Normal sodium hydroxide. Heat in a
gravity displacement autoclave (pressure) at 131 degrees for 30 minutes.
Take it out and clean it, and do routine sterilization for a double process to
get rid of the prions.
Second method : immerse in 1 normal sodium hypochlorite for 1 hour. Then
rinse them, autoclave at 121 degrees for 1 hour. Clean it the person routine
sterilization.
Third option: immerse it in either solution for 1 hour. Then remove it, place
in an open pan, autoclave at 134 for 1 hour, clean then do routine
sterilization.

Risk Control