The effect of prions on the brain in Albertan animals and people | 

Arroy (AJ) Jacob, Web Editor | 

Neurodegenerative diseases are rare, but they’re fast and fatal. For example, Albertans have seen 145 cases of Creutzfelt-Jakob disease (CJD) since 1998. Diseases like CJD are essential to keep track of because there is still no cure. However, more awareness of how these diseases occur at the molecular level never hurt an Albertan more than not knowing why their family members, pets, or cattle catch the disease to begin with.

Therefore, it’s essential to start with its origin—prions, now considered one of the most dangerous molecules on Earth.

What are prions?

Prions are proteins that already exist in healthy mammalian brains. The danger, however, comes from when one of these prions sporadically misfolds and “infects” all other proteins around it to do the same. Misfolding refers to the proteins twisting into a non-functional form. If more proteins in the brain lose their function, brain cell death occurs, and neurodegenerative diseases are diagnosed.

Various types of prion diseases have plagued mammals for a very long time. These diseases affect both animals and humans and can potentially transfer between species. This is possible because prions can survive in the environment for years.

Prion diseases in animals

Bovine spongiform encephalopathy (BSE), also known as “mad cow disease,” occurs in cattle. It mainly begins when they consume feed infected with prions. As the prions infect their brains and propagate protein misfolding, the cattle experience intense behavioural changes, including aggression and tremors. Physiological changes include loss of weight and milk production ability.

Chronic wasting disease (CWD), also known as “zombie deer disease,” plagues cervids (deer). It is more rare but is also just as fatal as BSE. Prions can infect a deer when it physically contacts an infected corpse or excrement. Like a virus, the prions can incubate within the new “vessel” for months before it exhibits symptoms. Some include excessive drooling, drinking and urination, and even depression before it dies in the wild. If another deer comes into contact with it, the cycle repeats. According to the Government of Alberta, the total number of CWD cases in the province since September 2005 is 6,163.

Prion diseases in humans

There is ample evidence to suggest that humans who consume beef that has been infected with prions develop variant Creutzfeldt-Jakob disease (CJD). This rare but invariably fatal neurodegenerative disorder overlaps with symptoms of dementia and physiological disrepair. Victims are bedridden and are given less than a year to survive before they are pulled into a permanent vegetative state. No studies suggest that consuming deer meat infected with prions would lead to human disease, but Alberta hunters, guides, and landowners are told to inform their local health authorities.

Despite its eeriness, prion-induced neurodegenerative diseases are infrequent amongst cattle, deer, and humans. Efforts since its discovery have kept the disease at bay. If anything regarding prions should be spread to the public, it should be its awareness.

Arroy (AJ) Jacob is the Web Editor for The Reflector 2024-2025. He is majoring in Cellular & Molecular Biology, and minoring in Creative Writing at Mount Royal University. He hopes to continue making scientific breakthroughs accessible, inclusive, and entertaining.