PRION BASICS Prion Definitions



Antibody: a protein that fights infections. Antibodies are a primary form of immune response in resistance to disease and act by attaching themselves to a foreign antigen (e.g. a bacteria or virus) and weakening or destroying it.


ALS: amyotrophic lateral sclerosis, commonly known as Lou Gehrig’s disease. ALS is a human protein misfolding disease.


Biomarkers: specific physical traits, including proteins and metabolites, that measure or indicate the presence, effects or progress of a disease.


BSE: bovine spongiform encephalopathy, commonly known as mad cow disease. BSE is a prion disease found in cattle.


CJD: Creutzfeldt-Jakob disease, a prion disease affecting humans.


CWD: chronic wasting disease, a prion disease found in cervids (deer, elk and moose).


Dementia: refers to a large class of disorders in which progressive brain damage leads to progressive deterioration of an individual’s ability to think (e.g. Alzheimer’s).


Incubation Period: the interval between the time an animal or human is infected with a disease and the appearance of symptoms.


In vitro: in an artificial environment (e.g. test tube).


In vivo: In a living organism.


Ruminant: an animal (like a cow or sheep) that has more than one stomach and swallows food and then brings it back up again to continue chewing it.


Prion: a misfolded protein found in mammals that causes infectious diseases of the nervous system in humans and animals.


Prion Protein: A normal protein present in the brains, spinal cords and many tissues of humans and other mammals. Its precise function is still unknown.


PrPC: Prion protein cellular. The normal function of prion protein is not entirely understood.


PrPSc: Prion protein scrapie. The abnormal, infectious prion that causes diseases in humans and animals.


Scrapie: a prion disease found in sheep and goats.


SRM: specified risk materials, parts of the cow that contain prions and might have the potential to transmit BSE. SRM includes the skull, brain, eyes, tonsils and spinal cord.


Transgenic: relating to or denoting an organism that contains genetic material into which DNA from an unrelated organism has been artificially introduced.


TSE: transmissible spongiform encephalopathy, also known as prion disease.


vCJD: variant Creutzfeldt-Jakob disease, a prion disease affecting humans that is acquired from eating BSE-infected material.


Zoonosis: a disease that can be transmitted from animals to humans.