Useful Terminology

Neurogenerative - a condition that results in or is characterised by the degeneration of the nervous

system especially the brain
Spontaneous - occurs suddenly and without protein
Protein - Lage molecules composed of one or more chains of amino acids joined together by peptide
bonds

What are prions?

• They are misfolded proteins that occur naturally in organisms
• When prions come in contact with healthy proteins they will change the conformation of the
protein and cause it to become misfolded as well
• They are associated with many neurodegenerative disease
• Prions do not have any RNA or DNA
Prion Structure
• They are entirely made out of proteins
• a misfold may cause the protein
Transmission
• They occur spontaneously in an indivual
• They can however be transmitted to others
• This is primarily through the ingestion of the misfolded proteins
• Mad Cow Disease
o Caused by farmers feeding their cattle meat from infected cows
Spontaneous Generation
• Most of time our body is prepared for dealing with misfolded proteins
• After a protein is made it is double checked for errors and any misfolded proteins are either
corrected or destroyed
Checking Errors
• The systems the body has for checking proteins can be worn away or have error themselves
• If this happens the prion is able to freely damage and misfold other proteins
• this can quickly domino out of control if it is not caught early enough
How do prions cause disease
• They can incubate within an indivual for a long time usually 5 to 20 years
• Prions will begin to build up in areas of the nervous system this can disrupt the normal tissue
function
• Diseases caused by prions are unable to be treated once symptoms begin to shows and are
100% fatal
Notable Prion Diseases
• Creutzfeldt-Jakob Disease - occurs in humans
• Scrapie - occurs in sheep and goats
• Mad Cow Disease - occurs in cattle
Creutzfeldt-Jakob Disease
• Occur in humans and over time will cause the degeneration of the nervous system
• When initial symptoms occur the condition will worsen over time sometime within months
or year
• Early symptoms
o Memory loss
o Poor coordination
o Visual disturbance
o Behaviour problems
• Late symptoms
o Blindness
 o Dementia
o Involuntary movements
o Weakness
o coma
• Around 70% of people die withing 1 year of diagnosis
• 85% of this disease occur for unknown reasons
• 7.5% of this due to genetic disorders inherited by parents

Scrapie
• Found in sheep and goats
• Name comes from the most noticeable symptom of the disease and horrible ich
o This causes the infected to scrape away their fleece causing damage to themselves
• This cannot be cured
• Can be spread among the herd very quickly
• Efforts are mostly focused on containing, quarantining and kill the infected indivual
Mad Cow Disease
• Early symptoms
o Trouble walking
o Weight loss
o Behavioural changes
• Late symptoms
o Inability to walk
o Organ failures
o Death
• Time between infection and initial onset of symptoms is usually 4-5 years
• Time between initial onset of symptoms and death is typically week to months